The DOI matches its Crossref record; 48 of the 48 references it cites were found in a registry or an index; 6 of the 38 sources its claims cite were checked for support.
Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy
Abstract
X-linked adrenoleukodystrophy (X-ALD) is a severe neurodegenerative disease caused by loss of function of the peroxisomal transporter ABCD1 (ALD), which results in accumulation of very long chain fatty acids (VLCFAs) in organs and serum, central demyelination and peripheral axonopathy and Addison's disease. Knockout of the ALD gene in the mouse (ALD(-)) results in an adrenomyeloneuropathy-like disease (a late onset form of X-ALD). In the present study, we demonstrate that axonal damage occurs as first pathological event in this model, followed by myelin degeneration. We show that this phenotype can be modulated through expression levels of an ALD-related gene (ALDR/ABCD2), its closest paralogue and a target of PPARalpha and SREBP transcription factors. Overexpression of ALDR in ALD(-) mice prevents both VLCFAs accumulation and the neurodegenerative features, whereas double mutants for ALD and ALDR exhibit an earlier onset and more severe disease (including signs of inflammatory reaction) when compared with ALD single mutants. Thus, our results provide direct evidence for functional redundancy/overlap between both transporters in vivo and highlight ALDR as therapeutic target for treatment of X-ALD.
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87 Own result · 16 No full text of the sources · 15 Own interpretation · 8 No basis given · 4 Own method · 3 Partly supported · 2 Supported so far · 2 Sources not found · 2 Not checked yet · accuracy not yet measured
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In the paper
“This striking absence of phenotype -genotype correlations suggests the existence of modifier genes, modifying environmental conditions and/or stochastic factors.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > INTRODUCTION
What the sources say
- Partly supportedcites [13] Kemp, S., Pujol, A., Waterham, H.R., van Geel, B.M., Boehm, C.D., Raymond, G.V., Cutting, G.R., Wanders, R.J. and Moser, H.W. (2001) ABCD1 mutations and the X-linked adrenoleukodystrophy mutation database: role in diagnosis and clinical correlations. Hum. Mutat., 18, 499–515. sourceExperimental — accuracy not yet measured
The GENOTYPEPHENOTYPE section reports a lack of correlation between ABCD1 mutations and disease severity, while the INTRODUCTION discusses proposed modifier genes and possible environmental factors. Neither passage proposes stochastic factors, so that part of the attributed explanation is unsupported by our copy; no page numbers are available. (LLM, conf=0.86)
- Not matchedcites [14] Moser, H., Smith, K.D., Watkins, P.A., Powers, J. and Moser, A.B. (2001) X-linked adrenoleukodystrophy. In Scriver, C. (ed.), The Metabolic and Molecular Bases of Inherited disease, 8th edn, McGraw-Hill, Vol. II, pp. 3303–3257.
Couldn’t be matched to a paper in Crossref, so it can’t be checked.
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In the paper
“However, the various forms of the disease may occur associated to the same mutation, even within the same family (13,14).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > INTRODUCTION
What the sources say
- Supportedcites [13] Kemp, S., Pujol, A., Waterham, H.R., van Geel, B.M., Boehm, C.D., Raymond, G.V., Cutting, G.R., Wanders, R.J. and Moser, H.W. (2001) ABCD1 mutations and the X-linked adrenoleukodystrophy mutation database: role in diagnosis and clinical correlations. Hum. Mutat., 18, 499–515. source
The Introduction (page not provided) says that different X-ALD phenotypes frequently co-occur within the same family and that males in one family may express different phenotypes. The Abstract also reports no apparent correlation between genotype and phenotype, supporting the same-mutation part of the claim. (LLM, conf=0.92)
- Not matchedcites [14] Moser, H., Smith, K.D., Watkins, P.A., Powers, J. and Moser, A.B. (2001) X-linked adrenoleukodystrophy. In Scriver, C. (ed.), The Metabolic and Molecular Bases of Inherited disease, 8th edn, McGraw-Hill, Vol. II, pp. 3303–3257.
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In the paper
“it is proposed that ABCD1 is implicated in the import of VLCFAs into the peroxisome (19,20).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > INTRODUCTION
What the sources say
- Not checked — no full text of the sourcecites [19] Hettema, E.H., van Roermund, C.W., Distel, B., van den Berg, M., Vilela, C., Rodrigues-Pousada, C., Wanders, R.J. and Tabak, H.F. (1996) The ABC transporter proteins Pat1 and Pat2 are required for import of long-chain fatty acids into peroxisomes of Saccharomyces cerevisiae. EMBO J., 15, 3813–3822. source
- Supportedcites [20] Shani, N., Watkins, P.A. and Valle, D. (1995) PXA1, a possible Saccharomyces cerevisiae ortholog of the human adrenoleukodystrophy gene. Proc. Natl Acad. Sci. USA, 92, 6012–6016. source
The Discussion states that involvement of ALDp in transporting very long-chain fatty acids into human peroxisomes is consistent with the biochemical phenotype of adrenoleukodystrophy. This supports the citing sentence’s proposed role, rather than establishing transport experimentally. (LLM, conf=0.91)
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In the paper
“So far, the only proven therapy available for X-ALD, at least the CCALD phenotype, is bone marrow transplantation (45,46).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > DISCUSSION
What the sources say
- Partly supportedcites [45] Aubourg, P., Blanche, S., Jambaque, I., Rocchiccioli, F., Kalifa, G., Naud-Saudreau, C., Rolland, M.O., Debre, M., Chaussain, J.L., Griscelli, C. et al. (1990) Reversal of early neurologic and neuroradiologic manifestations of X-linked adrenoleukodystrophy by bone marrow transplantation. N. Engl. J. Med., 322, 1860–1866. sourceExperimental — accuracy not yet measured
The paper reports improvement after bone marrow transplantation in one child with early cerebral disease, while the Discussion (p. 6) says this single case supports further evaluation. It does not establish transplantation as a proven therapy or the only available therapy; the Bone Marrow Transplantation section (p. 3) explicitly calls its benefits unproved. (LLM, conf=0.88)
- Not checked — no full text of the sourcecites [46] Shapiro, E., Krivit, W., Lockman, L., Jambaque, I., Peters, C., Cowan, M., Harris, R., Blanche, S., Bordigoni, P., Loes, D. et al. (2000) Long-term effect of bone-marrow transplantation for childhood-onset cerebral X-linked adrenoleukodystrophy. Lancet, 356, 713–718. source
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In the paper
“Overexpression of ALDR in ALD 2 mice prevents both VLCFAs accumulation and the neurodegenerative features”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Abstract
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In the paper
“Overexpression of ALDR in ALD 2 mice prevents both VLCFAs accumulation and the neurodegenerative features”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Abstract
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“We show that this phenotype can be modulated through expression levels of an ALD-related gene (ALDR/ABCD2)”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Abstract
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“including signs of inflammatory reaction”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Abstract
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In the paper
“whereas double mutants for ALD and ALDR exhibit an earlier onset and more severe disease (including signs of inflammatory reaction) when compared with ALD single mutants.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Abstract
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“Accumulation of VLCFAs in ALD 2 deficient peripheral nerves had not yet been described; we found it to be in a similar range than in spinal cord (around 6-fold).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > ALDR expression levels modulate the ALD phenotype in the mouse
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“However, one animal did show mild lesions, similar to those found in double mutants at 12 months of age (Table 3).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Spinal cord histopathology.
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“At 12 months of age, and consistent with our previous results (15), we found that ALD 2 mice had no altered SNCV or CMAP waves.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Electrophysiology of the PNS.
What the sources say
- Not checked — no full text of the sourcecites [15] Pujol, A., Hindelang, C., Callizot, N., Bartsch, U., Schachner, M. and Mandel, J.L. (2002) Late onset neurological phenotype of the X-ALD gene inactivation in mice: a mouse model for adrenomyeloneuropathy. Hum. Mol. Genet., 11, 499–505. source
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In the paper
“ALDR mutants showed no detectable abnormality at this precise age.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Electrophysiology of the PNS.
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In the paper
“This normalization of the biochemical phenotype correlates with a striking improvement of the neurological AMN-like phenotype presented by ALD 2 mice”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > INTRODUCTION
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“Myelin debris is a prevalent feature as revealed with Sudan black staining (Fig. 2M -P).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Spinal cord histopathology.
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“However, in the adrenal gland, we observed a 2-fold accumulation of C24:0 and C26:0 for ALDR 2/2”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > ALDR expression levels modulate the ALD phenotype in the mouse
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In the paper
“Remarkably, all pathological features are prevented upon ALDR overexpression (ALD 2 /tgALDR mice at 22 months of age, n ¼ 4 mice out of 5 analysed).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Spinal cord histopathology.
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“Overexpression of the ALDR protein prevented the onset of this pathological feature (Fig. 1D)”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Adrenal gland histopathology.
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“These needle-like structures were not found in 12-or 20-month-old ALD 2 /tgALDR animals.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Adrenal gland histopathology.
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“The absence of ALDR protein in central or peripheral nervous system did not seem to affect levels of VLCFAs.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > ALDR expression levels modulate the ALD phenotype in the mouse
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“In 8-month-old mice, we found that ALD inactivation leads to elevated levels of C26:0 in spinal cord and adrenal gland in agreement with previous reports (31 -33).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > ALDR expression levels modulate the ALD phenotype in the mouse
What the sources say
- Not checked — no full text of the sourcecites [31] Forss-Petter, S., Werner, H., Berger, J., Lassmann, H., Molzer, B., Schwab, M.H., Bernheimer, H., Zimmermann, F. and Nave, K.A. (1997) Targeted inactivation of the X-linked adrenoleukodystrophy gene in mice. J. Neurosci. Res., 50, 829–843. source
- Not checked — no full text of the sourcecites [32] Lu, J.F., Lawler, A.M., Watkins, P.A., Powers, J.M., Moser, A.B., Moser, H.W. and Smith, K.D. (1997) A mouse model for X-linked adrenoleukodystrophy. Proc. Natl Acad. Sci. USA, 94, 9366–9371. source
- Not checked — no full text of the sourcecites [33] Kobayashi, T., Shinnoh, N., Kondo, A. and Yamada, T. (1997) Adrenoleukodystrophy protein-deficient mice represent abnormality of very long chain fatty acid metabolism. Biochem. Biophys. Res. Commun., 232, 631–636. source
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In the paper
“Mice overexpressing the ALDR protein on wildtype background (ALD þ /tgALDR) were analysed and no difference with respect to control littermates was detected in 20-month-old animals (Table 2).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Electrophysiology of the PNS.
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“In serum, double mutants showed a significantly higher accumulation of C26:0 levels than single ALD or ALDR mutants (P , 0.01).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > ALDR expression levels modulate the ALD phenotype in the mouse
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“whereas the double mutants showed even higher levels of C24:0 and C26:0 than for ALD 2 or ALDR 2/2 .”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > ALDR expression levels modulate the ALD phenotype in the mouse
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“Most importantly, overexpression of the ALDR protein in the ALD 2 background normalized VLCFA levels in all organs analysed.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > ALDR expression levels modulate the ALD phenotype in the mouse
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“Our results demonstrate an accelerated and more severe neurological phenotype in the double KO mice”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > INTRODUCTION
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In the paper
“The inactivation of the murine ALDR gene by gene targeting (classical homologous recombination), as described elsewhere (A.Pujol et al., unpublished data), leads to total absence of the protein as shown by western blotting (Fig. 1A).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Generation of ALDR overexpressing and double ALD/ALDR mutant mice
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“Furthermore, we noted a marked tendency to lowering levels of C24:0 and C26:0 upon ALDR overexpression in a ALD þ background.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > ALDR expression levels modulate the ALD phenotype in the mouse
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“Consistent with previous reports (30), transgene expression was detected in all organs analysed (brain, spinal cord, adrenal gland, kidney, sciatic nerve, skeletal muscle, intestine and skin).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Generation of ALDR overexpressing and double ALD/ALDR mutant mice
What the sources say
- Not checked — no full text of the sourcecites [30] Okabe, M., Ikawa, M., Kominami, K., Nakanishi, T. and Nishimune, Y. (1997) ‘Green mice’ as a source of ubiquitous green cells. FEBS Lett., 407, 313–319. source
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“On semi-thin sections, adrenals of 18-month-old ALD 2 mice exhibited pronounced fibrosis of the reticular and fascicular cell layers (Fig. 1C, arrows).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Adrenal gland histopathology.
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In the paper
“One line was selected on the basis of the higher expression levels of the ALDR protein in the main target organs of interest: spinal cord, sciatic nerve and adrenal gland (5 -10fold overexpression when compared with wild-type levels, Fig. 1A).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Generation of ALDR overexpressing and double ALD/ALDR mutant mice
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“whereas double knockout presented a dramatic accumulation of lipid droplets across the adrenal cortex, encompassing glomerular, fascicular and reticular cell layers (Fig. 1E).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Adrenal gland histopathology.
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“Overexpression of ALDR protein prevented the phenotype due to the lack of functional ALDP, regarding both motor and sensitive waves.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Electrophysiology of the PNS.
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In the paper
“Immunoreactivity against amyloid precursor protein (APP, Fig. 2I -L) and synaptophysin (Fig. 2Q -T) is often found along axonal swellings, strongly suggesting axonal damage.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Spinal cord histopathology.
boostedcorrelational- Asserted: passes — The authors put it forward as true.
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In the paper
“In the current work, we describe that stable overexpression of ALDRP in the ALD 2 mouse background leads to full correction of VLCFA levels in target organs.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > INTRODUCTION
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In the paper
“This is accompanied by lipidic debris of myelin, as revealed with Sudan black, arrows in (N) and (O).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Immunohistochemistry
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The paper states this without a source or data behind it here, so there is nothing to check it against. The quote shows it is in the paper.
In the paper
“It is remarkable that, in spite of a high degree of functional redundancy between ALD and ALDR, ALD-mice (that express ALDR at normal, endogenous levels) do show phenotype.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > DISCUSSION
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In the paper
“No expression of ALDR protein is detected in ALDR 2/2 mice, whereas the transgenic line (tg) shows expression levels of ALDR 5-10-fold higher than wild-type littermates.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Immunohistochemistry
plain- Asserted: passes — The authors put it forward as true.
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In the paper
“Prevention of lesions occurs in ALD 2 /tgALDR mice (D, H, L, P, T, X).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Immunohistochemistry
plain- Asserted: passes — The authors put it forward as true.
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In the paper
“APP deposits are seen in axons in ALD 2 and ALD 2 /ALDR 2/2 mice, arrows in (J) and (K).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Immunohistochemistry
plainn = 2- Asserted: passes — The authors put it forward as true.
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In the paper
“Upon ALDR overexpression, these deficits were significantly prevented (Fig. 3E and G), to almost normal levels.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Spinal cord histopathology.
plaindirect- Asserted: passes — The authors put it forward as true.
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In the paper
“Furthermore, by routine haematoxylin -eosin and toluidin blue stainings on semi-thin sections, we detected a few inflammatory cell infiltrates in double mutant animals, which were not found in either single ALD 2 or ALDR 2/2 mice.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Spinal cord histopathology.
plain- Asserted: passes — The authors put it forward as true.
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In the paper
“No abnormalities were found in 22-monthold mice overexpressing ALDR (ALD þ /tgALDR).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Spinal cord histopathology.
plain- Asserted: passes — The authors put it forward as true.
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In the paper
“Furthermore, we show that 5-10-fold overexpression of ALDR in a broad range of tissues is not detrimental to the mouse as far as we could analyse.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > DISCUSSION
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In the paper
“Reactive astrogliosis is observed in ALD 2 and ALD 2 /ALDR 2/2 mice when compared with wt (A-C and E-g).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Immunohistochemistry
plaincorrelational- Asserted: passes — The authors put it forward as true.
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In the paper
“Indeed, ALD þ /tgALDR and wt/tgALDR mice behaved like control littermates in all neurological, electrophysiological and histopathological tests described in the present manuscript.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > DISCUSSION
plain- Asserted: passes — The authors put it forward as true.
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In the paper
“Our results unequivocally demonstrate that overexpression of ALDR can fully correct the ALDdependent accumulation of VLCFAs in the mouse tissues analysed.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > DISCUSSION
boosteddirect- Asserted: passes — The authors put it forward as true.
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In the paper
“A synergistic effect between ALD and ALDR is also seen in serum, since only double mutants show significantly higher accumulation of C26:0.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > DISCUSSION
plaindirect- Asserted: passes — The authors put it forward as true.
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In the paper
“In the nervous system of our models, a complex correlation between biochemical and pathological findings was observed.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > DISCUSSION
plaincorrelational- Asserted: passes — The authors put it forward as true.
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A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“Analysis of 12-month-old sections revealed that the first and most reliable marker of pathology was found to be synaptophysin-immunoreactive deposits in axonal swellings of ALD 2 and ALDR 2/2 mice, and to a larger extent in double mutant mice.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Spinal cord histopathology.
plaincorrelational- Asserted: passes — The authors put it forward as true.
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A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“Performance in the rotarod was considerably worsened for double mutant animals, which exhibited severe impairment already at 15 months of age (Fig. 3D).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Spinal cord histopathology.
plaindirect- Asserted: passes — The authors put it forward as true.
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A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“The more severe pathology in the CNS and PNS of the double KO mouse contrast with the finding of equivalent levels of VLCFAs in the nervous system, compared with the single KO mice.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > DISCUSSION
plaincorrelational- Asserted: passes — The authors put it forward as true.
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A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“No similar findings are found in WT mice (I, M).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Immunohistochemistry
plain- Asserted: passes — The authors put it forward as true.
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The paper states this without a source or data behind it here, so there is nothing to check it against. The quote shows it is in the paper.
In the paper
“Furthermore, double knockouts present inflammatory infiltrates in the spinal cord composed mainly of T lymphocytes, pathological features that are not present in single ALD or ALDR mutants.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > DISCUSSION
plain- Asserted: passes — The authors put it forward as true.
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A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“Abnormal fibres filled with synaptophysin are found in ALD 2 and 2KO mice (R, S).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Immunohistochemistry
plaincorrelational- Asserted: passes — The authors put it forward as true.
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- Load-bearing: unclear — It is unclear whether the paper’s conclusions depend on it.
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A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“Despite the degenerative changes and astrogliosis, there was no evidence of increased apoptosis, as estimated by the biotinylated UTP nick end labelling method and caspase-3 (17 kDa) immunohistochemistry.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Spinal cord histopathology.
hedged- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: unclear — It is unclear whether the paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“(E) Double mutants exhibit a greater accumulation of lipid droplets in fascicular and reticular cell layers.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Immunohistochemistry
plaincorrelational- Asserted: passes — The authors put it forward as true.
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A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“Inflammatory cells are found in perivascular, perimeningeal and Virchow-Robin spaces.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Immunohistochemistry
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: unclear — It is unclear whether the paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“Two sets of ALD þ /tgALDR mice (at 12 and 20 months of age, n ¼ 8 and 9, respectively) were tested in the open field and rotarod, scoring similar than their wild-type littermates (data not shown).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Spinal cord histopathology.
plain- Asserted: passes — The authors put it forward as true.
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- Load-bearing: passes — The paper’s conclusions depend on it.
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A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“This rescue of the biochemical phenotype is translated into a striking long-term prevention of the neurodegenerative features due to ALD loss of function, up to 20 -22 months of age.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > DISCUSSION
boosteddirect- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“No expression of ALDR protein is detected in ALDR 2/2 mice, whereas the transgenic line (tg) shows expression levels of ALDR 5-10-fold higher than wild-type littermates.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Immunohistochemistry
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
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A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“(F) Cholesterol needles are a characteristic feature found in lysosomes of ALD mutants.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Immunohistochemistry
plaincorrelational- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: unclear — It is unclear whether the paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“This would be in agreement with higher expression levels of ALDR in adrenals [Fig.1A and quantitative (Q)-PCR, data not shown].”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > DISCUSSION
hedgedcorrelational- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: unclear — It is unclear whether the paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“We have indeed searched (by Q-PCR) for an eventual compensatory upregulation of ALDR in ALD-mice but found no differences with respect to wild-type littermates.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > DISCUSSION
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
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The paper states this without a source or data behind it here, so there is nothing to check it against. The quote shows it is in the paper.
In the paper
“One useful aspect of the double knockouts is that the AMNlike pathology is more severe with an earlier onset (12 months rather than 20 months in the ALD KO).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > DISCUSSION
plaincorrelational- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“Electron micrographs reveals a cluster of T lymphocytes at the Virchow-Robin space.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Immunohistochemistry
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: unclear — It is unclear whether the paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“ALD 2 are different from wt (P , 0.001), ALD 2 /tgALDR and ALD 2 /ALDR 2/2 (P , 0.01).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Immunohistochemistry
plaincorrelationaln = 16- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“ALDR 2/2 (n ¼ 9) | 1.02 + 0.04 | 39.93 + 7.43 | 54.01 + 5.27”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Immunohistochemistry
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
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A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“ALD 2 (n ¼ 5) | 1.10 + 0.15 | 2.24 + 0.20 à | 0.82 + 0.27 à | 0.74 + 0.26 Ô
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Immunohistochemistry
plaincorrelational- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“In a perivascular infiltrate, the majority of cells are CD3þ positive T cells.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Immunohistochemistry
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: unclear — It is unclear whether the paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“(F) Number of rearings at 20 months ( Ã P , 0.01 when compared with wt).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Immunohistochemistry
plaincorrelationaln = 16- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: unclear — It is unclear whether the paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
A conclusion the paper draws from its own results — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“Thus, our results provide direct evidence for functional redundancy/overlap between both transporters in vivo”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Abstract
boostedcorrelational- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
In the paper
“Knockout of the ALD gene in the mouse (ALD 2 ) results in an adrenomyeloneuropathy-like disease (a late onset form of X-ALD).”
- Also stated in: § INTRODUCTION · plain
“A mouse model of the disease (Abcd1 knockout mouse, here called ALD 2 ) also accumulates VLCFAs in target organs, and has recently been shown to develop an AMN-like phenotype (15).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Abstract
What the sources say
- Not checked — no full text of the sourcecites [15] Pujol, A., Hindelang, C., Callizot, N., Bartsch, U., Schachner, M. and Mandel, J.L. (2002) Late onset neurological phenotype of the X-ALD gene inactivation in mice: a mouse model for adrenomyeloneuropathy. Hum. Mol. Genet., 11, 499–505. source
plaindirect- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
- Also stated in: § INTRODUCTION · plain
In the paper
“X-linked adrenoleukodystrophy (X-ALD) is a severe neurodegenerative disease caused by loss of function of the peroxisomal transporter ABCD1 (ALD)”
- Also stated in: § INTRODUCTION · plain
“The disease is caused by point mutations or deletions in the ALD (ABCD1) gene that inactivate the ALD protein (ALDP).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Abstract
What the sources say
- Not matchedcites [14] Moser, H., Smith, K.D., Watkins, P.A., Powers, J. and Moser, A.B. (2001) X-linked adrenoleukodystrophy. In Scriver, C. (ed.), The Metabolic and Molecular Bases of Inherited disease, 8th edn, McGraw-Hill, Vol. II, pp. 3303–3257.
Couldn’t be matched to a paper in Crossref, so it can’t be checked.
plaindirect- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
- Also stated in: § INTRODUCTION · plain
A conclusion the paper draws from its own results — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“highlight ALDR as therapeutic target for treatment of X-ALD.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Abstract
plaindirect- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
In the paper
“and the ability of ALDR to complement the biochemical defect in X-ALD fibroblasts when overexpressed (22).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > INTRODUCTION
What the sources say
- Not checked — no full text of the sourcecites [22] Netik, A., Forss-Petter, S., Holzinger, A., Molzer, B., Unterrainer, G. and Berger, J. (1999) Adrenoleukodystrophy-related protein can compensate functionally for adrenoleukodystrophy protein deficiency (X-ALD): implications for therapy. Hum. Mol. Genet., 8, 907–913. source
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In the paper
“The two paralogues are differentially expressed in a mirror-like pattern, especially in brain (23).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > INTRODUCTION
What the sources say
- Not checked — no full text of the sourcecites [23] Troffer-Charlier, N., Doerflinger, N., Metzger, E., Fouquet, F., Mandel, J.L. and Aubourg, P. (1998) Mirror expression of adrenoleukodystrophy and adrenoleukodystrophy related genes in mouse tissues and human cell lines. Eur. J. Cell Biol., 75, 254–264. source
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In the paper
“consistent with the concept of a high degree of overlapping functions between the two transporters.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > INTRODUCTION
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In the paper
“Thus, our data strengthen the potential of stimulating ALDR expression as feasible approach to X-ALD treatment.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > INTRODUCTION
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In the paper
“However, this study fell short of discriminating between whether the effect on VLCFAs was due to increased levels of ALDR, and/or to the concomitant peroxisome proliferation, or other pleiotropic effects caused by the drug, such as the correction of mitochondrial dysfunction (29).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > INTRODUCTION
What the sources say
- Not checked — no full text of the sourcecites [29] McGuinness, M.C., Lu, J.F., Zhang, H.P., Dong, G.X., Heinzer, A.K., Watkins, P.A., Powers, J. and Smith, K.D. (2003) Role of ALDP (ABCD1) and mitochondria in X-linked adrenoleukodystrophy. Mol. Cell. Biol., 23, 744–753. source
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In the paper
“The ALD gene encodes a peroxisomal ABC transporter protein (ALDP), which belongs to a small family of peroxisomal transporters that includes three other members: ABCD2 or ALDR (ALD-related protein) (16), ABCD3 or PMP70 (17) and ABCD4 or PMP70R (18).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > INTRODUCTION
What the sources say
- Not checked — no full text of the sourcecites [16] Lombard-Platet, G., Savary, S., Sarde, C.O., Mandel, J.L. and Chimini, G. (1996) A close relative of the adrenoleukodystrophy (ALD) gene codes for a peroxisomal protein with a specific expression pattern. Proc. Natl Acad. Sci. USA, 93, 1265–1269. source
- Not checked — no full text of the sourcecites [17] Kamijo, K., Taketani, S., Yokota, S., Osumi, T. and Hashimoto, T. (1990) The 70-kDa peroxisomal membrane protein is a member of the Mdr (P- glycoprotein)-related ATP-binding protein superfamily. J. Biol. Chem., 265, 4534–4540. source
- Not checked — no full text of the sourcecites [18] Holzinger, A., Kammerer, S. and Roscher, A.A. (1997) Primary structure of human PMP69, a putative peroxisomal ABC-transporter. Biochem. Biophys. Res. Commun., 237, 152–157. source
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In the paper
“Thus, the two transporters share overlapping functions with respect to accumulation of C24:0 and C26:0 fatty acids.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > ALDR expression levels modulate the ALD phenotype in the mouse
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In the paper
“The possible functional equivalence of ALD and ALDR has been repeatedly suggested on the basis of their high sequence similarity (88% similarity and 67% identity at the protein level) (16)”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > INTRODUCTION
What the sources say
- Not checked — no full text of the sourcecites [16] Lombard-Platet, G., Savary, S., Sarde, C.O., Mandel, J.L. and Chimini, G. (1996) A close relative of the adrenoleukodystrophy (ALD) gene codes for a peroxisomal protein with a specific expression pattern. Proc. Natl Acad. Sci. USA, 93, 1265–1269. source
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In the paper
“demonstrating that ALDR can compensate for the absence of ALD.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > INTRODUCTION
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In the paper
“since upregulation of ALDR would substitute for the defective function of the ALD gene right at the first step of the pathological cascade, the biochemical dysfunction.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > DISCUSSION
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In the paper
“Gene transfer of ALDR could also circumvent this inconvenience, as is the case for utrophin in mdx (dystrophin deficient) muscles (47).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > DISCUSSION
What the sources say
- Not checked — no full text of the sourcecites [47] Cerletti, M., Negri, T., Cozzi, F., Colpo, R., Andreetta, F., Croci, D., Davies, K.E., Cornelio, F., Pozza, O., Karpati, G. et al. (2003) Dystrophic phenotype of canine X-linked muscular dystrophy is mitigated by adenovirus-mediated utrophin gene transfer. Gene Ther., 10, 750–757. source
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In the paper
“Thus, the functional redundancy of ALD and ALDR that was observed in cell culture systems is indeed operating in vivo.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > DISCUSSION
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In the paper
“This fact suggests that precise tissue-specific upregulation may not be necessary for therapeutic benefit to patients.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > DISCUSSION
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- Rests on an assumption: Findings in the ALD 2 mouse model and its modulation by ALDR can be extrapolated to human X-ALD patients.
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In the paper
“ALDR overexpression is thus a plausible therapeutic strategy, as it prevents the whole panoplies of alterations caused by ALD loss in the mouse, from the biochemical abnormalities to the pathological and neurological manifestations.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > DISCUSSION
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In the paper
“indicating a relevant functional interaction between the two transporters.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > DISCUSSION
hedgedcorrelational- Asserted: passes — The authors put it forward as true.
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In the paper
“Definitive proof pointing at VLCFA accumulation as pathogenesis-causative in CNS is lacking at present”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > DISCUSSION
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- It may state two separate claims as one. 37
- Probably not a substantial claim. 11
- The quote only partly states this claim. 1
The sources with a verdict support this claim; the rest weren’t checked yet, have no full text we could read, or their verdicts aren’t rated yet.
In the paper
“although elevated levels of VLCFA have been shown to be detrimental to adrenocortical cells in culture (34,42) and to model membranes (43).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > DISCUSSION
What the sources say
- Not checked yetcites [34] Powers, J.M. (1985) Adreno-leukodystrophy (adreno-testiculo-leukomyelo-neuropathic-complex). Clin. Neuropathol., 4, 181–199.
- Supportedcites [42] Whitcomb, R.W., Linehan, W.M. and Knazek, R.A. (1988) Effects of long-chain, saturated fatty acids on membrane microviscosity and adrenocorticotropin responsiveness of human adrenocortical cells in vitro. J. Clin. Invest., 81, 185–188. source
The Results report that human adrenocortical cells cultured with hexacosanoic or lignoceric acid had reduced basal and ACTH-stimulated cortisol responses and increased membrane microviscosity. This supports the adrenocortical-cell part of the citing sentence; the separate model-membrane part is attributed to citation 43, not this paper. (LLM, conf=0.93)
- Supportedcites [43] Ho, J.K., Moser, H., Kishimoto, Y. and Hamilton, J.A. (1995) Interactions of a very long chain fatty acid with model membranes and serum albumin. Implications for the pathogenesis of adrenoleukodystrophy. J. Clin. Invest., 96, 1455–1463. source
Citation (43) is attached to the model-membrane portion of the sentence, not the cultured adrenocortical-cell portion. The Discussion reports that excess VLCFA profoundly affects artificial membrane structure and describes its effect on membrane structure and function as deleterious. (LLM, conf=0.92)
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In the paper
“In the present study, we demonstrate that axonal damage occurs as first pathological event in this model, followed by myelin degeneration.”
- Also stated in: § Spinal cord histopathology. · plain
“Thus, axonal degeneration precedes myelin disturbances in our mouse models, resembling findings in AMN patients (35).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Abstract
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- Also stated in: § Spinal cord histopathology. · plain
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In the paper
“At 20 months, ALD mutants exhibit slowing of both motor and sensitive conduction velocities.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Electrophysiology of the PNS.
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In the paper
“A very similar type of pathology is found in ALDR 2/2 (Table 3), and in a more severe manner, in double mutant mice.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Spinal cord histopathology.
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In the paper
“Those lesions are ubiquitinated (Fig. 2U -X), but they do not colocalize with myelin lesions as shown by serial sections analysis.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Spinal cord histopathology.
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In the paper
“At 22 months, ALD 2 mice present signs of microglia and astrocyte activation, numerous lectin-positive cells with morphological characteristics of ramified microglia/macrophages, and glial fibrillary acidic protein (GFAP) positive cells (Fig. 2A -H).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Spinal cord histopathology.
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In the paper
“Interestingly, the C22:0 metabolite accumulated specifically in ALDR 2/2 adrenals and reached a 4-fold increase in double mutants.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > ALDR expression levels modulate the ALD phenotype in the mouse
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In the paper
“All mice described were born at expected Mendelian ratios and developed to maturity without showing any abnormal clinical signs.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Generation of ALDR overexpressing and double ALD/ALDR mutant mice
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In the paper
“The absence of ALDR expression does not seem to influence these parameters, since ALDR 2/2 mice show normal waves pattern, and double mutants do not have more important abnormalities than ALD 2 mice at this age.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Electrophysiology of the PNS.
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In the paper
“On the contrary, the latency of motor wave and the SNCV were slowed down in double mutants (Table 2).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Electrophysiology of the PNS.
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In the paper
“At the ultrastructural level, we observed in the ALD 2 mice (in particular in the fascicular cell layer of cortex and cortico-medullary junction), intralysosomal needle-like cholesterol inclusions, which are also seen in macrophages of ALD patients (34) (Fig. 1F).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Adrenal gland histopathology.
What the sources say
- Not checked — no full text of the sourcecites [34] Powers, J.M. (1985) Adreno-leukodystrophy (adreno-testiculo-leukomyelo-neuropathic-complex). Clin. Neuropathol., 4, 181–199.
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In the paper
“In ALD 2 / ALDR 2/2 mice, however, these inclusions were spread all over the cytoplasm, and are encountered 10 times more often than in ALD 2 mice (Fig. 1G).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Adrenal gland histopathology.
plaincorrelational- Asserted: passes — The authors put it forward as true.
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In the paper
“ALDR 2/2 animals were viable, fertile and showed no overt pathology or behavioural abnormalities until at least 6 months of age.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Generation of ALDR overexpressing and double ALD/ALDR mutant mice
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In the paper
“(G) In double mutants, the cholesterol needles are not restricted to lysosomes, but are scattered all over the cytoplasm and are much more abundant.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Immunohistochemistry
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In the paper
“At 20 months of age, ALD 2 mice presented an impairment of their locomotor coordination in the rotarod test (Fig. 3E) and exploratory abilities in the open field test (Fig. 3G).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Spinal cord histopathology.
plaincorrelational- Asserted: passes — The authors put it forward as true.
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In the paper
“Double mutants exhibit the same range of VLCFA accumulation than ALD mutants (at least at 8 months of age) in spite of a more severe and earlier onset neurological phenotype at the histological, behavioural and electrophysiological levels.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > DISCUSSION
plain- Asserted: passes — The authors put it forward as true.
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None of the sources cited for this claim have been checked yet.
In the paper
“APP and synaptophysin deposits are ubiquitinated (V, W).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Immunohistochemistry
plain- Asserted: passes — The authors put it forward as true.
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Probably not a substantial claim. The paper’s conclusions may not depend on it.
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In the paper
“ALDR fully compensates for ALD loss upon overexpression, but its inactivation does not cause VLCFA accumulation in whole homogenates of CNS (brain and spinal cord) or PNS.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > DISCUSSION
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In the paper
“In the open field, double mutants behaved even less explorative than ALD 2 mice (P , 0.01), and had a marked tendency to freezing (long latency before starting the movement) (Fig. 3G).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Spinal cord histopathology.
plaincorrelational- Asserted: passes — The authors put it forward as true.
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In the paper
“(C) ALD mutants show fibrosis at the corticomedular junction (arrows) that is prevented through ALDR overexpression in (D).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Immunohistochemistry
plaindirect- Asserted: passes — The authors put it forward as true.
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In the paper
“Consistently, in double knockouts we found a synergistic accumulation of C22:0, C24:0 and C26:0, which correlated with a very marked increase in numbers of speckles and cholesterol needles, together with a more dramatic accumulation of lipid droplets in virtually all adrenal cortex cell layers (fascicular, reticular and glomerular)”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > DISCUSSION
plaincorrelational- Asserted: passes — The authors put it forward as true.
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In the paper
“Those infiltrates were typically in small clusters (10 -20 cells) adjacent to blood vessels and perimeningeal areas, and were composed mainly of T lymphocytes (Fig. 3A -C).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Spinal cord histopathology.
plain- Asserted: passes — The authors put it forward as true.
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In the paper
“In contrast, in adrenals, ALDR inactivation results in an increase of C22:0 (unlike ALD knockouts) and C24:0 fatty acids (similar levels than in ALD 2 mice).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > DISCUSSION
plaindirect- Asserted: passes — The authors put it forward as true.
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In the paper
“ALD (n ¼ 9) ALD 2 /tgALDR | 1.23 + 0.05 Ã 0.99 + 0.06 | 32.90 + 2.73 Ã 46.41 + 4.34 | 42.40 + 4.33 ÃÃ 53.60 + 3.14”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Immunohistochemistry
plain- Asserted: passes — The authors put it forward as true.
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In the paper
“ALD 2/2 tg ALDR (n ¼ 5) | 8.01 + 1.35 | 9.71 + 1.03 | 0.39 + 0.12 Ãà | 0.05 + 0.02 ÃÔ
- Also stated in: § Immunohistochemistry · plain
“ALD 2 /tgALDR (n ¼ 2) | 15.96 + 2.40 † | 16.86 + 2.59 ÃÃ, ‡ | 0.11 + 0.01 ÃÃ, † ‡ | 0.01 + 0.00 ÃÔ
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Immunohistochemistry
plaindirect- Asserted: passes — The authors put it forward as true.
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- Also stated in: § Immunohistochemistry · plain
A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“ALD 2 /ALDR 2/2 | 1.12 + 0.01 à | 42.18 + 11.23 | 44.53 + 6.55 ÃÔ
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Immunohistochemistry
plaindirect- Asserted: passes — The authors put it forward as true.
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A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“ALD 2 (n ¼ 5) | 11.92 + 1.83 | 28.72 + 3.57 | 3.80 + 0.57 à | 0.32 + 0.03 Ô
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Immunohistochemistry
plaincorrelational- Asserted: passes — The authors put it forward as true.
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In the paper
“ALD 2 (n ¼ 8) ALDR 2/2 (n ¼ 4) | 7.54 + 1.78 6.93 + 1.40 7.92 + 1.25 | 10.38 + 1.65 11.22 + 2.56 7.62 + 0.97 | 0.37 + 0.07 2.46 + 0.66 Ã 0.69 + 0.23 | 0.05 + 0.01 0.36 + 0.05 Ã 0.09 + 0.04 |”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Immunohistochemistry
plaincorrelational- Asserted: passes — The authors put it forward as true.
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It may state two separate claims as one.
A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“ALD (n ¼ 9) ALD 2 /tgALDR | 1.23 + 0.05 Ã 0.99 + 0.06 | 32.90 + 2.73 Ã 46.41 + 4.34 | 42.40 + 4.33 ÃÃ 53.60 + 3.14”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Immunohistochemistry
plaincorrelational- Asserted: passes — The authors put it forward as true.
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In the paper
“ALD 2 /ALDR 2/2 | 1.19 + 0.02 à | 33.85 + 6.02 à | 43.51 + 5.23 ÃÔ
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Immunohistochemistry
plaincorrelational- Asserted: passes — The authors put it forward as true.
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In the paper
“which results in accumulation of very long chain fatty acids (VLCFAs) in organs and serum, central demyelination and peripheral axonopathy and Addison's disease.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Abstract
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A claim is checked only against the cited paper’s full text, never its abstract, and none is available for its sources.
In the paper
“Functional gene redundancy is a widespread mechanism in vertebrate evolution, thought to increase robustness against deleterious mutations by 'backing-up' vital functions for the cell or the organism (1,2).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > INTRODUCTION
What the sources say
- Not checked — no full text of the sourcecites [1] Thomas, J.H. (1993) Thinking about genetic redundancy. Trends Genet., 9, 395–399. source
- Not checked — no full text of the sourcecites [2] Gu, Z., Steinmetz, L.M., Gu, X., Scharfe, C., Davis, R.W. and Li, W.H. (2003) Role of duplicate genes in genetic robustness against null mutations. Nature, 421, 63–66. source
plainconditional- Asserted: passes — The authors put it forward as true.
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Probably not a substantial claim.
In the paper
“X-ALD patients accumulate very long chain fatty acids (VLCFA) in plasma and tissues, notably in the adrenal cortex and nervous system.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > INTRODUCTION
What the sources say
- Not matchedcites [14] Moser, H., Smith, K.D., Watkins, P.A., Powers, J. and Moser, A.B. (2001) X-linked adrenoleukodystrophy. In Scriver, C. (ed.), The Metabolic and Molecular Bases of Inherited disease, 8th edn, McGraw-Hill, Vol. II, pp. 3303–3257.
Couldn’t be matched to a paper in Crossref, so it can’t be checked.
plain- Asserted: passes — The authors put it forward as true.
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Probably not a substantial claim. The paper’s conclusions may not depend on it.
- Not matched
A claim is checked only against the cited paper’s full text, never its abstract, and none is available for its sources.
In the paper
“its closest paralogue and a target of PPARa and SREBP transcription factors.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Abstract
What the sources say
- Not checked — no full text of the sourcecites [24] Berger, J., Albet, S., Bentejac, M., Netik, A., Holzinger, A., Roscher, A.A., Bugaut, M. and Forss-Petter, S. (1999) The four murine peroxisomal ABC-transporter genes differ in constitutive, inducible and developmental expression. Eur. J. Biochem., 265, 719–727. source
- Not checked — no full text of the sourcecites [25] Fourcade, S., Savary, S., Albet, S., Gauthe, D., Gondcaille, C., Pineau, T., Bellenger, J., Bentejac, M., Holzinger, A., Berger, J. et al. (2001) Fibrate induction of the adrenoleukodystrophy-related gene (ABCD2): promoter analysis and role of the peroxisome proliferator-activated receptor PPARalpha. Eur. J. Biochem., 268, 3490–3500. source
- Not checked — no full text of the sourcecites [28] Weinhofer, I., Forss-Petter, S., Zigman, M. and Berger, J. (2002) Cholesterol regulates ABCD2 expression: implications for the therapy of X-linked adrenoleukodystrophy. Hum. Mol. Genet., 11, 2701–2708. source
plaincorrelational- Asserted: passes — The authors put it forward as true.
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It may state two separate claims as one.
The paper states this without a source or data behind it here, so there is nothing to check it against. The quote shows it is in the paper.
In the paper
“In a human genetic disease, a related gene whose function overlaps with the defective gene function may be considered as therapeutic target.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > INTRODUCTION
hedgedconditional- Asserted: passes — The authors put it forward as true.
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Probably not a substantial claim. It may be too vague to check against evidence.
In the paper
“Dietary treatment with 4PBA in ALD 2 mice has been shown to partially correct the accumulation of VLCFAs in brain and adrenal glands, correlating with ALDR protein induction and increased peroxisome proliferation (27).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > INTRODUCTION
What the sources say
- Not checked — no full text of the sourcecites [27] Kemp, S., Wei, H.M., Lu, J.F., Braiterman, L.T., McGuinness, M.C., Moser, A.B., Watkins, P.A. and Smith, K.D. (1998) Gene redundancy and pharmacological gene therapy: implications for X-linked adrenoleukodystrophy. Nat. Med., 4, 1261–1268. source
plaindirect- Asserted: passes — The authors put it forward as true.
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It may state two separate claims as one.
What the paper did — it cites no source for this, so there is none to check. The quote shows it is in the paper.
In the paper
“Histology, morphometry, behavioural testing and electrophysiological experiments were performed in a blind way with respect to the animal's genotype.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Mouse breeding
plain- Asserted: passes — The authors put it forward as true.
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Probably not a substantial claim. The paper’s conclusions may not depend on it.
In the paper
“Infiltrating T lymphocytes (CD8 þ ) are often found in unaffected white matter of ALD and AMN/ALD patients, and also in acute demyelinative lesions together with macrophages (44).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > DISCUSSION
What the sources say
- Not checked — no full text of the sourcecites [44] Ito, M., Blumberg, B.M., Mock, D.J., Goodman, A.D., Moser, A.B., Moser, H.W., Smith, K.D. and Powers, J.M. (2001) Potential environmental and host participants in the early white matter lesion of adreno-leukodystrophy: morphologic evidence for CD8 cytotoxic T cells, cytolysis of oligodendrocytes, and CD1-mediated lipid antigen presentation. J. Neuropathol. Exp. Neurol., 60, 1004–1019. source
plaincorrelational- Asserted: passes — The authors put it forward as true.
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It may state two separate claims as one.
A conclusion the paper draws from its own results — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“Alternatively and most likely, specificity of transported substrates is overlapping to some degree, but not identical between the two transporters.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > DISCUSSION
hedgedcorrelational- Asserted: passes — The authors put it forward as true.
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It may state two separate claims as one.
A conclusion the paper draws from its own results — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“Thus, this suggests that double knockouts may be useful to analyse the mechanisms of the inflammatory reaction that plays a major role in CCALD pathology in humans.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > DISCUSSION
hedgedconditional- Asserted: passes — The authors put it forward as true.
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Probably not a substantial claim.
In the paper
“Thus, axonal degeneration precedes myelin disturbances in our mouse models, resembling findings in AMN patients (35).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Spinal cord histopathology.
What the sources say
- Not checked — no full text of the sourcecites [35] Powers, J.M., DeCiero, D.P., Ito, M., Moser, A.B. and Moser, H.W. (2000) Adrenomyeloneuropathy: a neuropathologic review featuring its noninflammatory myelopathy. J. Neuropathol. Exp. Neurol., 59, 89–102. source
plaincorrelational- Asserted: passes — The authors put it forward as true.
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It may state two separate claims as one.
A conclusion the paper draws from its own results — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“Thus, ALDR would be less efficient at substituting for ALD's function, and its overexpression would therefore be required to reach therapeutic benefit.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > DISCUSSION
hedgeddirect- Asserted: passes — The authors put it forward as true.
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It may state two separate claims as one.
None of the sources cited for this claim have been checked yet.
In the paper
“Plasma VLCFA levels in patients are not related to phenotype onset or severity, nor does their normalization through a dietary treatment (Lorenzo's oil, a mixture of erucic and oleic acids) seems to be curative or prevents disease onset (14,41).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > DISCUSSION
What the sources say
- Not matchedcites [14] Moser, H., Smith, K.D., Watkins, P.A., Powers, J. and Moser, A.B. (2001) X-linked adrenoleukodystrophy. In Scriver, C. (ed.), The Metabolic and Molecular Bases of Inherited disease, 8th edn, McGraw-Hill, Vol. II, pp. 3303–3257.
Couldn’t be matched to a paper in Crossref, so it can’t be checked.
- Not checked — no full text of the sourcecites [41] Aubourg, P., Adamsbaum, C., Lavallard-Rousseau, M.C., Rocchiccioli, F., Cartier, N., Jambaque, I., Jakobezak, C., Lemaitre, A., Boureau, F., Wolf, C. et al. (1993) A two-year trial of oleic and erucic acids (‘Lorenzo's oil’) as treatment for adrenomyeloneuropathy. N. Engl. J. Med., 329, 745–752. source
hedged- Asserted: passes — The authors put it forward as true.
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It may state two separate claims as one.
- Not matched
The paper states this without a source or data behind it here, so there is nothing to check it against. The quote shows it is in the paper.
In the paper
“Many reasons can account for this fact, for instance that the endogenous ALDR gene is 5-10 times less expressed in physiological conditions than in the transgenic mice, and its normal expression pattern is not ubiquitous, being predominantly expressed in neurons in the CNS.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > DISCUSSION
hedged- Asserted: passes — The authors put it forward as true.
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Probably not a substantial claim. The paper may give no reason why it holds. It may state two separate claims as one.
A conclusion the paper draws from its own results — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“Altogether, and within the limits of potential interspecies differences, our findings warrant further efforts aiming at upregulation of ALDR as a therapy for X-ALD.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > DISCUSSION
hedgedconditional- Asserted: passes — The authors put it forward as true.
- Checkable: fails — It may be too vague to check against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
Probably not a substantial claim. It may be too vague to check against evidence.
- Rests on an assumption: Findings in the ALD 2 mouse model and its modulation by ALDR can be extrapolated to human X-ALD patients.
What the paper did — it cites no source for this, so there is none to check. The quote shows it is in the paper.
In the paper
“At least three sections corresponding to the dorsal columns of the spinal cord were analysed per animal and per stain.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Immunohistochemistry
plain- Asserted: passes — The authors put it forward as true.
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Probably not a substantial claim. The paper’s conclusions may not depend on it.
What the paper did — it cites no source for this, so there is none to check. The quote shows it is in the paper.
In the paper
“Crucial experiments (rotarod, open field tests and EMG) were confirmed with pure C57BL/6J background animals.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Mouse breeding
plain- Asserted: passes — The authors put it forward as true.
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- Based: passes — The paper gives a reason why it holds.
Probably not a substantial claim. The paper’s conclusions may not depend on it.
What the paper did — it cites no source for this, so there is none to check. The quote shows it is in the paper.
In the paper
“Double heterozygous were intercrossed to obtain double knockout mice and wild-type littermate controls; the offspring obeyed mendelian ratios.”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Mouse breeding
plain- Asserted: passes — The authors put it forward as true.
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- Load-bearing: fails — The paper’s conclusions may not depend on it.
- Based: passes — The paper gives a reason why it holds.
Probably not a substantial claim. The paper’s conclusions may not depend on it.
The paper relies on this without support.
Findings in the ALD 2 mouse model and its modulation by ALDR can be extrapolated to human X-ALD patients.
“Altogether, and within the limits of potential interspecies differences, our findings warrant further efforts aiming at upregulation of ALDR as a therapy for X-ALD.”
“This fact suggests that precise tissue-specific upregulation may not be necessary for therapeutic benefit to patients.”
Claims other papers make about this one
Each is checked against this paper’s own text: does it say what the citing paper says it does?
The cited source doesn’t support the claim as written.
In the citing paper
“Interestingly, Acbd1–/– mice, despite high VLCFA accumulation, do not develop cerebral disease and instead model the milder peripheral adrenomyelopathy, with late-onset symptoms appearing around 20 months of age, whereas Acbd2–/– mice did not develop symptoms (Pujol et al., 2004).”
From The neurological pathology of peroxisomal ACBD5 deficiency – lessons from patients and mouse models (2025) · cites this paper as Pujol et al., 2004
Checked by a reasoning model — may be wrong.
Experimental — accuracy not yet measuredThe source backs part of the claim, not all of it — or backs it for a different population, condition or comparison.
In the citing paper
“It is intriguing that also Abcd1/Abcd2 double-deficient mice do not develop brain inflammation or demyelination [156], in spite of the finding that Abcd1/Abcd2 double-deficient peritoneal macrophages are metabolically much more severely affected than those from single transporter-deficient mice [157]. Also in these mice, the neuropathology is restricted mainly to axonopathy in the spinal cord and,”
From Peroxisomes in brain development and function (2016) · cites this paper as [156]
Experimental — accuracy not yet measuredThe source backs part of the claim, not all of it — or backs it for a different population, condition or comparison.
In the citing paper
“One notable exception are the dorsal root ganglia [33], which may explain the earlier onset of a sensory motor phenotype in Abcd1/Abcd2 double-deficient and Abcd2 single-deficient mice compared with Abcd1-deficient mice [31].”
From Abcd2 Is a Strong Modifier of the Metabolic Impairments in Peritoneal Macrophages of Abcd1-Deficient Mice (2014) · cites this paper as [31]
Experimental — accuracy not yet measuredThe source backs part of the claim, not all of it — or backs it for a different population, condition or comparison.
In the citing paper
“Abcd1: Abcd2 double KO mice had an earlier onset of symptoms with more severe motor deficits (at 20 months of age, 6 out of 11 mice were not able to perform motor tests), axonal degeneration, and myelin alterations, but no cerebral pathology.”
From Revisiting the Pathogenesis of X-Linked Adrenoleukodystrophy (2025) · cites this paper as [190]
Experimental — accuracy not yet measuredThe source backs part of the claim, not all of it — or backs it for a different population, condition or comparison.
In the citing paper
“Histological abnormalities in spinal cord dorsal and ventral columns became evident at 20 months, while atrophy or death of Purkinje cells was detected in the cerebellum.”
From Revisiting the Pathogenesis of X-Linked Adrenoleukodystrophy (2025) · cites this paper as [190]
Experimental — accuracy not yet measured
The source backs part of the claim, not all of it — or backs it for a different population, condition or comparison.
In the citing paper
“Electron microscopy in Abcd2 KO mice showed the first signs of spinal cord axonal pathology at 12 months, yet no histological abnormalities.”
From Revisiting the Pathogenesis of X-Linked Adrenoleukodystrophy (2025) · cites this paper as [190]
Experimental — accuracy not yet measuredThe source backs part of the claim, not all of it — or backs it for a different population, condition or comparison.
In the citing paper
“At 12 months of age, Abcd2 KO mice already displayed a marked impairment in their motor performance, which aggravated up to 20 months.”
From Revisiting the Pathogenesis of X-Linked Adrenoleukodystrophy (2025) · cites this paper as [190]
Experimental — accuracy not yet measuredThe source backs part of the claim, not all of it — or backs it for a different population, condition or comparison.
In the citing paper
“Upon overexpression, ABCD2101–104 and, to some extent, ABCD35,102,105 can compensate for the lack of ABCD1 in vivo and in vitro, and therefore represent excellent candidates for a modulation of the phenotype.”
From The genetic landscape of X-linked adrenoleukodystrophy: inheritance, mutations, modifier genes, and diagnosis (2015) · cites this paper as [103]
Experimental — accuracy not yet measuredThe source backs part of the claim, not all of it — or backs it for a different population, condition or comparison.
In the citing paper
“It has been reported that, upon overexpression, both of these homologs are in principle able to compensate for dysfunctional ABCD1.5,101–103”
From The genetic landscape of X-linked adrenoleukodystrophy: inheritance, mutations, modifier genes, and diagnosis (2015) · cites this paper as [103]
Experimental — accuracy not yet measuredThe source backs part of the claim, not all of it — or backs it for a different population, condition or comparison.
In the citing paper
“These molecular differences are also reflected in vivo: Acbd1–/– mice exhibit a roughly sixfold increase in C26:0 levels in the spinal cord—approximately three times higher than in Acbd5–/– mice—whereas Acbd2–/– mice show no such accumulation (Pujol et al., 2004; Granadeiro et al., 2024).”
From The neurological pathology of peroxisomal ACBD5 deficiency – lessons from patients and mouse models (2025) · cites this paper as Pujol et al., 2004
Experimental — accuracy not yet measuredThe source backs part of the claim, not all of it — or backs it for a different population, condition or comparison.
In the citing paper
“Interestingly, Acbd1–/– mice, despite high VLCFA accumulation, do not develop cerebral disease and instead model the milder peripheral adrenomyelopathy, with late-onset symptoms appearing around 20 months of age, whereas Acbd2–/– mice did not develop symptoms (Pujol et al., 2004).”
From The neurological pathology of peroxisomal ACBD5 deficiency – lessons from patients and mouse models (2025) · cites this paper as Pujol et al., 2004
Experimental — accuracy not yet measuredThe cited source says what the claim attributes to it.
In the citing paper
“The extent of VLCFA accumulation in myelin-enriched tissues, such as spinal cord and sciatic nerve, was reported to be five to sixfold in Abcd1-deficient mice and was not further elevated in Abcd1/Abcd2 double-deficient mice [31].”
From Abcd2 Is a Strong Modifier of the Metabolic Impairments in Peritoneal Macrophages of Abcd1-Deficient Mice (2014) · cites this paper as [31]
The cited source says what the claim attributes to it.
In the citing paper
“Inactivation of Abcd2 alone has no significant effect on the levels of saturated VLCFA in most murine tissues including brain, spinal cord and sciatic nerve, which are affected by the pathology in X-ALD [31], which is also the case in our Abcd2 −/− mouse strain (Forss-Petter, unpublished observation).”
From Abcd2 Is a Strong Modifier of the Metabolic Impairments in Peritoneal Macrophages of Abcd1-Deficient Mice (2014) · cites this paper as [31]
The cited source says what the claim attributes to it.
In the citing paper
“inactivation of abcd2 does not lead to accumulation of VLCFAs in nervous tissues”
From Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage (2005) · cites this paper as [18]
The cited source says what the claim attributes to it.
In the citing paper
“its stable overexpression by transgenesis in the ALD knoc-kout mouse prevents accumulation of VLCFAs as well as the onset of neurological phenotype”
From Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage (2005) · cites this paper as [18]
The cited source says what the claim attributes to it.
In the citing paper
“These molecular differences are also reflected in vivo: Acbd1–/– mice exhibit a roughly sixfold increase in C26:0 levels in the spinal cord—approximately three times higher than in Acbd5–/– mice—whereas Acbd2–/– mice show no such accumulation (Pujol et al., 2004; Granadeiro et al., 2024).”
From The neurological pathology of peroxisomal ACBD5 deficiency – lessons from patients and mouse models (2025) · cites this paper as Pujol et al., 2004
The check wasn’t confident either way, so it gives no verdict — never disputed.
In the citing paper
“However, as also Abcd1/Abcd2 double-deficient mice do not develop demyelinating brain inflammation (Forss-Petter, unpublished observation) and [31], additional species differences or other unknown triggers must play a critical role.”
From Abcd2 Is a Strong Modifier of the Metabolic Impairments in Peritoneal Macrophages of Abcd1-Deficient Mice (2014) · cites this paper as [31]
Experimental — accuracy not yet measuredThe check wasn’t confident either way, so it gives no verdict — never disputed.
In the citing paper
“The brain of Abcd2 KO mice showed no significant difference compared to wild type littermates.”
From Revisiting the Pathogenesis of X-Linked Adrenoleukodystrophy (2025) · cites this paper as [190]
Experimental — accuracy not yet measuredIn the citing paper
“Using transgenic expression of Abcd2 in the Abcd1 knock-out mouse, Pujol et al. demonstrated that VLCFA accumulation and disease phenotype could be corrected in vivo [ 40 ].”
From Peroxisomal ABC Transporters: An Update (2021) · cites this paper as [40]
In the citing paper
“Abcd1/Abcd2 double knock-out mice have an earlier and more severe neurological phenotype associated with inflammatory T lymphocyte infiltration in the spinal cord [ 40 ]”
From Peroxisomal ABC Transporters: An Update (2021) · cites this paper as [40]
In the citing paper
“the biochemical and neurological defects observed in the Abcd1 knock-out mice can be corrected by ubiquitous transgenic expression of Abcd2 [ 40 ]”
From Peroxisomal ABC Transporters: An Update (2021) · cites this paper as [40]
In the citing paper
“In the spinal cord, inflammation is observed in old mice and includes microglia and astrocyte activation [ 40 ]”
From Peroxisomal ABC Transporters: An Update (2021) · cites this paper as [40]
Self-check
Percentages, sums and reported differences re-computed from the paper’s own numbers.
Each contradiction between two passages listed here was confirmed by an independent check.
Rated minor: it would not touch a result — a value no result rests on, a p-value that stays on the same side of the significance level, or a conflict that is clerical, resolved elsewhere in the paper, or outside its own findings.
In the paper
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Adrenal gland histopathology.
“On semi-thin sections, adrenals of 18-month-old ALD 2 mice exhibited pronounced fibrosis of the reticular and fascicular cell layers (Fig. 1C, arrows).”
§ Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy > Immunohistochemistry
“(B -G) Histopathology of adrenal gland at 12 months.”
Citations
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Efficient selection for high-expression transfectants with a novel eukaryotic vector
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‘Green mice’ as a source of ubiquitous green cells
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Role of duplicate genes in genetic robustness against null mutations
Gu, Z., Steinmetz, L. M., Gu, X., Scharfe, C., Davis, R. W., & Li, W.-H. (2003) · Nature · doi:10.1038/nature01198 · Cited by 889
Moser, H. W., Mahmood, A., & Raymond, G. V. (2007) · Nature Clinical Practice Neurology · doi:10.1038/ncpneuro0421 · Cited by 663
Kamijo, K., Taketani, S., Yokota, S., Osumi, T., & Hashimoto, T. (1990) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(19)39595-x · Cited by 391
Brichta, L. (2003) · Human Molecular Genetics · doi:10.1093/hmg/ddg256 · Cited by 382
Aubourg, P., Blanche, S., Jambaqué, I., Rocchiccioli, F., Kalifa, G., Naud-Saudreau, C., Rolland, M.-O., Debré, M., Chaussain, J.-L., Griscelli, C., et al. (1990) · New England Journal of Medicine · doi:10.1056/nejm199006283222607 · Cited by 366
Shapiro, E., Krivit, W., Lockman, L., Jambaqué, I., Peters, C., Cowan, M., Harris, R., Blanche, S., Bordigoni, P., Loes, D., et al. (2000) · The Lancet · doi:10.1016/s0140-6736(00)02629-5 · Cited by 329
Adrenoleukodystrophy: Incidence, new mutation rate, and results of extended family screening
Bezman, L., Moser, A. B., Raymond, G. V., Rinaldo, P., Watkins, P. A., Smith, K. D., Kass, N. E., & Moser, H. W. (2001) · Annals of Neurology · doi:10.1002/ana.101 · Cited by 324
Hettema, E. H., van Roermund, C. W., Distel, B., van den Berg, M., Vilela, C., Rodrigues‐Pousada, C., Wanders, R. J., & Tabak, H. F. (1996) · The EMBO Journal · doi:10.1002/j.1460-2075.1996.tb00755.x · Cited by 323
Research works printed but not linked: 1
- Silva-Zolezzi, I., Hebron, K., Mihalik, S., Thomas, G., Valle, D. and Jimenez-Sanchez, G. (2003) Abcd3−/− mice have a non-shivering thermogenesis defect related to a disturbance in fasting fuel homeostasis. 53rd Annual Meeting of The American Society of Human Genetics. The American Society of Human Genetics, Los Angeles, California, Vol. 73, (suppl.) p. 199.
Cited by
Sorted by citations
Biochemistry of mammalian peroxisomes revisited
Wanders, R. J. A., & Waterham, H. R. (2006) · Annual Review of Biochemistry · doi:10.1146/annurev.biochem.74.082803.133329 · Cited by 995
Moser, H. W., Mahmood, A., & Raymond, G. V. (2007) · Nature Clinical Practice Neurology · doi:10.1038/ncpneuro0421 · Cited by 663
Chemotherapy-induced resistance by ATP-binding cassette transporter genes
Gillet, J. A., Efferth, T., & Remacle, J. (2007) · Biochimica et Biophysica Acta (BBA) - Reviews on Cancer · doi:10.1016/j.bbcan.2007.05.002 · Cited by 342
Pathophysiology of X-linked adrenoleukodystrophy
Berger, J., Forss-Petter, S., & Eichler, F. S. (2014) · Biochimie · doi:10.1016/j.biochi.2013.11.023 · Cited by 238
“By transgenic ubiquitous overexpression of ABCD2, the biochemical defect and the neurological phenotype of the Abcd1deficient mice could be rescued [45].”
BackgroundRole of ATP‐binding cassette transporters in brain lipid transport and neurological disease
Kim, W. S., Weickert, C. S., & Garner, B. (2007) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.2007.05099.x · Cited by 252
“In vitro and in vivo studies using Abcd1 knockout mice have shown that over-expression of ABCD2 (ALDRP) and ABCD3 (PMP70) can overcome the biochemical defects in VLCFA b-oxidation and prevent the onset of disease symptoms (Pujol et al. 2004).”
Background
Metadata
- Authors
- Pujol, A., Ferrer, I., Camps, C., Metzger, E., Hindelang, C., Callizot, N., Ruiz, M., Pàmpols, T., Giròs, M., & Mandel, J. L.
- Affiliations
- Centre National de la Recherche Scientifique (FR)
- Sig Holding (Switzerland) (CH)
- Corresponding author
- Pujol, Aurora
- Venue
- Human Molecular Genetics
- Publisher
- Oxford University Press (OUP)
- Published
- 2004-10-15
- Volume
- 13
- Issue
- 23
- Pages
- 2997-3006
- ISSN
- 0964-6906 (ISSN-L)
- 1460-2083
- OpenAlex
- W2104564418
- Cited by
- 201
- References
- 48
- Citations by year
- 2026: 2 · 2025: 5 · 2024: 10 · 2023: 9 · 2022: 11 · 2021: 7 · 2020: 11 · 2019: 7 · 2018: 6 · 2017: 10 · 2016: 6 · 2015: 8 · 2014: 12 · 2013: 14 · 2012: 10
- FWCI
- 7.23
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Human Molecular Genetics (publishedVersion)
- PubMed (publishedVersion)
- HAL (submittedVersion)
- HighWire Press Open Archive (submittedVersion)
- Funders
- European Commission
- Centre National de la Recherche Scientifique
- Association Européenne contre les Leucodystrophies
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
- Health Sciences › Medicine › Genetics (clinical) › Lysosomal Storage Disorders Research
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Hereditary Neurological Disorders
- Keywords
- X-linked adrenoleukodystrophy, ABCD1, ABCD2, very long chain fatty acids, functional redundancy, axonal injury, myelin degeneration, adrenomyeloneuropathy, PPARα, SREBP, therapeutic target, peroxisome biogenesis disorders
- MeSH terms
- ATP Binding Cassette Transporter, Subfamily D, ATP Binding Cassette Transporter, Subfamily D, Member 1, Adrenal Glands, Adrenoleukodystrophy, Animals, Humans, Mice, Mutant Strains, Mice, Transgenic, Microscopy, Electron, Phenotype, Spinal Cord, ATP-Binding Cassette Transporters, Mice
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
A language model drew the claims from the paper on 2026-10-11, under version 3 of the claim definition (model deepseek-v4.1-flash, prompt extract-v3).
A second model labelled each claim on 2026-10-11 (model jev-1.13-20260917, definition revision gold-guideline-v0.2).
Cited sources are checked with a language model’s help (gpt-6-sol).
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
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