The DOI matches its Crossref record; 58 of the 58 references it cites were found in a registry or an index; 3 of the 8 sources its claims cite were checked for support.
Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage
Open accessAbstract
ATP-binding cassette (ABC) transporters facilitate unidirectional translocation of chemically diverse substances, ranging from peptides to lipids, across cell or organelle membranes. In peroxisomes, a subfamily of four ABC transporters (ABCD1 to ABCD4) has been related to fatty acid transport, because patients with mutations in ABCD1 (ALD gene) suffer from X-linked adrenoleukodystrophy (X-ALD), a disease characterized by an accumulation of very-long-chain fatty acids (VLCFAs). Inactivation in the mouse of the abcd1 gene leads to a late-onset neurodegenerative condition, comparable to the late-onset form of X-ALD [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.]. In the present work, we have generated and characterized a mouse deficient for abcd2, the closest paralog to abcd1. The main pathological feature in abcd2-/- mice is a late-onset cerebellar and sensory ataxia, with loss of cerebellar Purkinje cells and dorsal root ganglia cell degeneration, correlating with accumulation of VLCFAs in the latter cellular population. Axonal degeneration was present in dorsal and ventral columns in spinal cord. We have identified mitochondrial, Golgi and endoplasmic reticulum damage as the underlying pathological mechanism, thus providing evidence of a disturbed organelle cross-talk, which may be at the…
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63 Own result · 16 Own interpretation · 4 No full text of the sources · 4 Own method · 3 Supported · 2 No basis given · 1 Sources not found
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“inactivation of abcd2 does not lead to accumulation of VLCFAs in nervous tissues”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > INTRODUCTION
What the sources say
- Supportedcites [18] Pujol, A., Ferrer, I., Camps, C., Metzger, E., Hindelang, C., Callizot, N., Ruiz, M., Pampols, T., Giros, M. and Mandel, J.L. (2004) Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy. Hum. Mol. Genet., 13, 2997–3006. source
The Discussion states that ALDR/ABCD2 inactivation does not cause VLCFA accumulation in whole homogenates of the mouse CNS or PNS. The results section likewise reports that absence of ALDR did not seem to affect VLCFA levels in the central or peripheral nervous system. (LLM, conf=0.93)
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“C24:0 levels are higher in double mutants than in wild-type or abcd1-fibroblasts (P , 0.01).”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Biochemical function of ALDRP
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In the paper
“Axonal degeneration was present in dorsal and ventral columns in spinal cord.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Abstract
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In the paper
“correlating with accumulation of VLCFAs in the latter cellular population”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Abstract
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“all the pathological changes noticed by ultrastructural or immunohistochemical techniques in abcd22/2 mice at DRGs, spinal cords and cerebellar sites were presented in the same extent, intensity and age by abcd1 deficient mice.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Biochemical function of ALDRP
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In the paper
“double mutant fibroblasts accumulate higher levels of C26:0 than abcd1-fibroblasts (P , 0.001).”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Biochemical function of ALDRP
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In the paper
“At 12 months of age, abcd22/2 mice present lower amplitude of the H-wave than their wild-type littermates”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Defective spinal reflex in abcd22/2 mice
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In the paper
“We observed disturbed endoplasmic reticulum and Golgi apparatus, with dilatation and fragmentation of channels and cisterns”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Biochemical function of ALDRP
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In the paper
“abcd22/2 null mice were significantly more active than their wild-type littermates.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Behavioral deficits in ALDRP-deficient mice
plaincorrelational- Asserted: passes — The authors put it forward as true.
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In the paper
“mice lacking ALDRP displayed progressive behavioral deficits, consistent with ongoing neural degeneration from 12 months onwards.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Behavioral deficits in ALDRP-deficient mice
plaindirect- Asserted: passes — The authors put it forward as true.
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In the paper
“In spinal cord dorsal and ventral columns, histological abnormalities not demonstrable at 12 months of age became evident in abcd22/2 mice at 20 months of age.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Biochemical function of ALDRP
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In the paper
“we detected gaps in the Purkinje cell monolayer in Nissl-stained sections”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Biochemical function of ALDRP
plain- Asserted: passes — The authors put it forward as true.
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In the paper
“Quantitative RT-PCR confirmed the absence of full-length abcd2 mRNA”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Generation of abcd2 null mutant mice (abcd22/2)
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In the paper
“The main pathological feature in abcd22/2 mice is a late-onset cerebellar and sensory ataxia”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Abstract
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
“with loss of cerebellar Purkinje cells”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Abstract
plaindirect- Asserted: passes — The authors put it forward as true.
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In the paper
“When compared with wild-type littermates at 12 months of age, abcd22/2 mice displayed a marked impairment in their performance during 4 days trial on the rotating rod set at an accelerating speed (P , 0.01 by ANOVA)”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Behavioral deficits in ALDRP-deficient mice
plaincorrelational- Asserted: passes — The authors put it forward as true.
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In the paper
“A significant number of mitochondria showed degenerative images and contained lipidic inclusions”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Biochemical function of ALDRP
plain- Asserted: passes — The authors put it forward as true.
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In the paper
“At 15 months of age, the time required for mice to reach a platform along a balance bar was significantly increased in abcd12/abcd22/2 double mutants (P , 0.001)”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Behavioral deficits in ALDRP-deficient mice
plaindirect- Asserted: passes — The authors put it forward as true.
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In the paper
“Absence of the abcd2 gene was not accompanied by compensatory increases of its closest paralog, the abcd1 gene”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Generation of abcd2 null mutant mice (abcd22/2)
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In the paper
“No difference could be demonstrated between mutants and controls”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Behavioral deficits in ALDRP-deficient mice
hedged- Asserted: passes — The authors put it forward as true.
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In the paper
“electron microscopy examination revealed a specific pattern of degeneration, more evident at 20 months of age.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Biochemical function of ALDRP
plaincorrelational- Asserted: passes — The authors put it forward as true.
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In the paper
“Western blots showed a complete absence of the protein in all tissues of abcd22/2 mice”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Generation of abcd2 null mutant mice (abcd22/2)
plain- Asserted: passes — The authors put it forward as true.
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In the paper
“dorsal root ganglia cell degeneration”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Abstract
plaindirect- Asserted: passes — The authors put it forward as true.
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In the paper
“We could indeed detect an accumulation of C26:0 in DRGs from abcd22/2 mice (Fig. 3I and J, P , 0.002).”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Biochemical function of ALDRP
hedgeddirect- Asserted: passes — The authors put it forward as true.
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In the paper
“Reactive astrogliosis is observed in aldr2/2 mice when compared with littermate controls.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Immunohistochemistry
plaincorrelational- Asserted: passes — The authors put it forward as true.
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In the paper
“In a ganglionar cell, mitochondria (m) with paracristallin profile (arrow) resulting from the juxtaposition of parallel-stacked cristae.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Immunohistochemistry
plainSingle case- Asserted: passes — The authors put it forward as true.
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In the paper
“Strong reduction of non-phosphorylated neurofilaments in Purkinje cells was seen by staining with anti-SMI32.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Immunohistochemistry
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In the paper
“High number of zebra bodies filling up a satellite cell (s).”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Immunohistochemistry
plain- Asserted: passes — The authors put it forward as true.
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In the paper
“Ald2 and double mutant fibroblasts show similar reduction to 60% of the wild-type level (P , 0.0008).”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Immunohistochemistry
plain- Asserted: passes — The authors put it forward as true.
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In the paper
“Loss of Purkinje cell somata evidenced by Nissl staining (arrowheads).”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Immunohistochemistry
plain- Asserted: passes — The authors put it forward as true.
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In the paper
“Decompactation of axolemma (arrow).”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Immunohistochemistry
plain- Asserted: passes — The authors put it forward as true.
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In the paper
“Quantification of saturated VLCFA levels by ESI-MS in adult fibroblasts and DRG samples, expressed as micromoles per milligram of protein.Wild-type levels have been set to 100.(Single asterisk) Significant difference versus wild-type.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Immunohistochemistry
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In the paper
“Synaptophysin and APP deposits are ubiquitinated (K and L).”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Immunohistochemistry
plain- Asserted: passes — The authors put it forward as true.
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In the paper
“Polymorphous residual and zebra-like bodies in cytoplasm of a ganglion cell (arrows).”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Immunohistochemistry
plain- Asserted: passes — The authors put it forward as true.
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In the paper
“Axonal sprouting within a large axon filled with neurofilaments (nf) surrounded by myelin sheath (ms), lipid droplet (ld).”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Immunohistochemistry
plainSingle case- Asserted: passes — The authors put it forward as true.
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In the paper
“APP deposits are seen in axonal swellings in aldr2/2 mice.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Immunohistochemistry
plain- Asserted: passes — The authors put it forward as true.
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In the paper
“Mitochondria containing lipidic residual bodies in satellite cells.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Immunohistochemistry
plain- Asserted: passes — The authors put it forward as true.
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In the paper
“An empty myelinated fiber containing two myelin bodies.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Immunohistochemistry
plainSingle case- 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
“Aldr2/2 slipped away more often than their wild-type littermates and ald-mice.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Immunohistochemistry
plaincorrelational- Asserted: passes — The authors put it forward as true.
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In the paper
“Open-field experiments showing lack of habituation of aldr2/2 mutants at 12 months of age (n ¼ 8 wt and n ¼ 12 aldr2/2).”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Immunohistochemistry
plainn = 12- Asserted: passes — The authors put it forward as true.
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In the paper
“This is accompanied by lipidic debris of myelin, as revealed with Sudan black in (G and H).”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Immunohistochemistry
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
“Dense, degenerating axoplasm focally surrounded by distorted myelin.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Immunohistochemistry
plain- Asserted: passes — The authors put it forward as true.
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In the paper
“Higher magnification of the region within the square in (C), showing tubulo-vesicular inclusions (tvi) and fragmented smooth ER (asterisk).”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > 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
“In (I and J), abnormal fibers filled with synaptophysin are found in aldr2/2 mice.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > 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
“Axonal sprouting consistent with axonal regeneration in sciatic nerve.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > 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 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
“which indicates that ALDRP is indeed able to take over from ALDP, at least when overexpressed.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > INTRODUCTION
hedgedconditional- 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
“In summary, abcd2 is widely expressed in different types of neuronal populations, from embryonic sensitive neurons of DRGs to mature differentiated spinal motor neurons.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Expression pattern of abcd1 and abcd2 genes
plain- 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.
- 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
“This finding is indicative of a lack of habituation capacity to a new environment.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Behavioral deficits in ALDRP-deficient mice
hedgeddirect- 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 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
“we present compelling evidence of a redundant biochemical function between the two transporters in the mouse.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > DISCUSSION
boostedcorrelational- 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 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
“These results indicate that the large proprioceptive sensory neurons at DRG or their afferents are functionally defective”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Defective spinal reflex in abcd22/2 mice
hedgeddirect- Asserted: passes — The authors put it forward as true.
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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, in DRGs, abcd2 seems to play the same role regarding C26:0 import/catabolism that abcd1 plays in most tissues.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Biochemical function of ALDRP
hedgeddirect- 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 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
“We have identified mitochondrial, Golgi and endoplasmic reticulum damage as the underlying pathological mechanism”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Abstract
plaindirect- Asserted: passes — The authors put it forward as true.
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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
“This suggests that abcd2 might cooperate or compensate to some extent for abcd1 loss of function.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Biochemical function of ALDRP
hedgedconditional- 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 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
“providing evidence of a disturbed organelle cross-talk, which may be at the origin”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Abstract
hedgedconditional- Asserted: passes — The authors put it forward as true.
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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, accumulation of VLCFAs in DRGs of abcd22/2 mice may increase the vulnerability to this particular cellular population to neurodegeneration.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > DISCUSSION
hedgedconditional- Asserted: passes — The authors put it forward as true.
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In the paper
“Because covalently bound fatty acids contribute to the tertiary structure of PLP in aqueous medium (50), this altered fatty acid composition may influence its physical or biological properties, compromising stability of PLP itself or its insertion in the myelin sheath.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > DISCUSSION
What the sources say
- Not checked — no full text of the sourcecites [50] Bizzozero, O.A. and Lees, M.B. (1986) Fatty acid acylation of rat brain myelin proteolipid protein in vitro: identification of the lipid donor. J. Neurochem., 46, 630–636. source
plaindirect- Asserted: passes — The authors put it forward as true.
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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
“Aldr2/2 mice present a progressive neurological phenotype.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Immunohistochemistry
plain- Asserted: passes — The authors put it forward as true.
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In the paper
“In adrenoleukodystrophy patients, the proportions of saturated VLCFAs bound to PLP have been found to be increased at the expense of oleic acid (49).”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > DISCUSSION
What the sources say
- Not checked — no full text of the sourcecites [49] Bizzozero, O.A., Zuniga, G. and Lees, M.B. (1991) Fatty acid composition of human myelin proteolipid protein in peroxisomal disorders. J. Neurochem., 56, 872–878. source
plaincorrelational- Asserted: passes — The authors put it forward as true.
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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
“The absence of H-wave is aldr dependent.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Immunohistochemistry
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.
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
“Southern blot analysis demonstrating homologous recombination at the Abcd2 locus in mouse tail genomic DNA.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Immunohistochemistry
boosted- 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.
- It may state two separate claims as one. 15
- Probably not a substantial claim. 16
- The quote only partly states this claim. 2
Every source the paper cites for this claim was checked and supports it.
In the paper
“its stable overexpression by transgenesis in the ALD knoc-kout mouse prevents accumulation of VLCFAs as well as the onset of neurological phenotype”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > INTRODUCTION
What the sources say
- Supportedcites [18] Pujol, A., Ferrer, I., Camps, C., Metzger, E., Hindelang, C., Callizot, N., Ruiz, M., Pampols, T., Giros, M. and Mandel, J.L. (2004) Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy. Hum. Mol. Genet., 13, 2997–3006. source
The Introduction describes stable ALDR/ABCD2 overexpression by transgenesis in ALD-knockout mice. The Results report normalized VLCFA levels, and the Discussion reports prevention of neurodegenerative features through 20–22 months of age. (LLM, conf=0.94)
plaindirect- 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.
It may state two separate claims as one.
- Supported
Every source the paper cites for this claim was checked and supports it.
In the paper
“Indeed, recent evidence indicates a severe impairment of fast axonal transport system in the PLP/DM20 deficient mouse (51), thus underscoring the importance of a healthy membrane for axonal function.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > DISCUSSION
What the sources say
- Supportedcites [51] Edgar, J.M., McLaughlin, M., Yool, D., Zhang, S.C., Fowler, J.H., Montague, P., Barrie, J.A., McCulloch, M.C., Duncan, I.D., Garbern, J. et al. (2004) Oligodendroglial modulation of fast axonal transport in a mouse model of hereditary spastic paraplegia. J. Cell Biol., 166, 121–131. source
The Results section, “Absence of PLP impairs fast axonal transport systems,” reports a retrograde-transport deficit in PLP/DM20-deficient mice that worsened with age; tracer fluorescence was only 31% of control after three days. The Discussion, “PLP/DM20 is necessary for fast transport in myelinated axons,” also states that both anterograde and retrograde transport are perturbed and that transport can arrest or become severely retarded. Page numbers were not supplied. (LLM, conf=0.93)
hedgeddirect- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- 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.
- Supported
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
“Mice lacking the abcd2 gene were delivered in expected Mendelian ratios, were viable and fertile and did not show any obvious abnormalities in development, behavior or gross morphology of the nervous system up to 6 months”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Generation of abcd2 null mutant mice (abcd22/2)
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.
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
“lack of ALDRP is neither translated into an accumulation of VLCFAs nor into a decrease of C26:0 b-oxidation capacity”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Biochemical function of ALDRP
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.
- Based: passes — The paper gives a reason why it holds.
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
“Double mutants showed a more severe involvement and earlier onset of pathological signs, evidenced from 12 months onwards.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Biochemical function of ALDRP
plaindirect- 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.
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
“the offspring obeyed Mendelian ratios.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Mouse generation, breeding and genotyping
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- 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.
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
“At the image center, enlargement of a non-myelinated fiber, with accumulation of organelles.Note myelinated degenerating axons, with decompactation of axolemma (asterisk).”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Immunohistochemistry
plainSingle case- 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.
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
“By the fourth trial, control animals ran the bar with not more than two slips.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Horizontal bar cross test
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- 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.
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 adult stages (weeks 5-6), aldr is expressed in interneurons of pons (G) and spinal cord (H).”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Immunohistochemistry
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- 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.
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 abcd2 -/ -mice were generated by inactivation of the abcd2 gene by classic homologous recombination.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Mouse generation, breeding and genotyping
plaindirect- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- 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.
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 contrast, the ald gene exhibits a low and ubiquitous expression pattern, throughout embryonic development (B and D) and adult pons (I) and spinal cord (J).”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Immunohistochemistry
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- 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.
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
“Axonal degeneration: involution of the cellular organites with the formation of a fibrillar body (f) and myelin-like bodies (arrow); numerous vacuoles.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > 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.
- Based: passes — The paper gives a reason why it holds.
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
“Motor neuron cell body with hypertrophy of abnormal Golgi (g) cisternae associated to fragmented smooth ER (asterisk).Mitochondrial enlargement and densification of cristae (arrows).”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > 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.
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
“In neurons, lamellar bodies derived from altered Golgi (g) and rough endoplasmic reticulum (arrows).Note mitochondria with tubular cristae (asterisk) and ring-shaped alteration (white arrow).”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > 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.
- Based: passes — The paper gives a reason why it holds.
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
“Focal loss of staining with anti-calbindin D28K in the aldr2/2 mice.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > 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.
The quote only partly states this claim.
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
“Double mutant animals show delayed time to cross the bar and increased number of slips.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Immunohistochemistry
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.
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
“Focal upregulations of GFAP expression in aldr2/2.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > 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.
The quote only partly states this claim.
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
“Proliferative neurons in cortex (C and E) and neuro-olfactive epitelium (F) are strongly positive for aldr at E14 and 5.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Immunohistochemistry
boostedcorrelational- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- 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.
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 is strongly expressed in DRGs at E11 and 5.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Immunohistochemistry
boosted- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- 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.
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 mitochondria with a lipid droplet and circular cristae in a ganglion cell (m).Fragmentation of Golgi cisternae (g).”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > 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.
- Based: passes — The paper gives a reason why it holds.
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
“Remnants of the Golgi apparatus in the form of lamellae (arrows), in a zone with small smooth ER vacuoles.Two lamellated bodies within an axonal fiber (open arrows).”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > 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.
- Based: passes — The paper gives a reason why it holds.
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
“We obtained three recombinant clones, out of 302, resistant to neomycin, which were injected into blastocysts from C57BL/6 mice.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Mouse generation, breeding and genotyping
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- 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.
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
“Myelin remnants in the Schwann cell, resulting in a fibrillar and granular body (f).Axolemma decompactation (arrow).”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > 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.
It may state two separate claims as one.
In the paper
“Inactivation in the mouse of the abcd1 gene leads to a late-onset neurodegenerative condition, comparable to the late-onset form of X-ALD”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Abstract
What the sources say
- Not matchedcites Pujol, A., Hindelang, C., Callizot, N., Bartsch, U., Schachner, M. and Mandel, J.L. (2002)
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: 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.
- Not matched
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
“Taking all pathological findings together, we conclude that abcd22/2 mice develop a lateonset spinocerebellar ataxia, with impaired propioception most likely due to neuronal degenerative processes at the DRG level or to synaptic defects in spinal cord.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > DISCUSSION
hedgedconditional- 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.
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
“Defective mitochondria may lead to a failure of ATP-dependent axoplasmic transport with consequent 'dying-back' axonal degeneration.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > DISCUSSION
hedgedconditional- Asserted: passes — The authors put it forward as true.
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- Based: passes — The paper gives a reason why it holds.
It may state two separate claims as one.
In the paper
“Mutations in ABCD1 results in X-linked adrenoleukodystrophy/adrenomyeloneuropathy (X-ALD/AMN) (12).”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > INTRODUCTION
What the sources say
- Not checked — no full text of the sourcecites [12] Mosser, J., Douar, A.M., Sarde, C.O., Kioschis, P., Feil, R., Moser, H., Poustka, A.M., Mandel, J.L. and Aubourg, P. (1993) Putative X-linked adrenoleukodystrophy gene shares unexpected homology with ABC transporters. Nature, 361, 726–730. source
plaindirect- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- 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.
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
“We propose that the composition, maintenance or recycling process of myelin and neuronal/axonal membrane might be disturbed in the ALD and ALDR models, leading to impaired axono-glia communication, which would be detrimental for both interconnected compartments.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > DISCUSSION
hedgedconditional- Asserted: passes — The authors put it forward as true.
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- Based: passes — The paper gives a reason why it holds.
It may state two separate claims as one.
In the paper
“VLCFAs levels have been found to be increased in X-ALD brain gangliosides (48), which might contribute to pathogenesis.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > DISCUSSION
What the sources say
- Not checked — no full text of the sourcecites [48] Igarashi, M., Schaumburg, H.H., Powers, J., Kishmoto, Y., Kolodny, E. and Suzuki, K. (1976) Fatty acid abnormality in adrenoleukodystrophy. J. Neurochem., 26, 851–860. source
plaincorrelational- 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.
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 underscoring the importance of a healthy membrane for axonal function.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > DISCUSSION
plaindirect- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- 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.
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 (locomotor tests and EMG) have been confirmed with pure C57BL/6J background mice.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Mouse generation, breeding and genotyping
boosted- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- 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.
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
“Results described in this manuscript are derived from mice on a mixed C57BL/ 6J/129Sv background (87% C57BL/6J and 13%129Sv).”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Mouse generation, breeding and genotyping
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- 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.
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, morphometric, behavioral testing and electrophysiological experiments were performed in a blind way with respect to the animal's genotype.”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Mouse generation, breeding and genotyping
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- 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.
No human disease has been attributed to defects in the genes coding for ALDRP (ABCD2), PMP70 (ABCD3) or PMP69 (ABCD4).
“To date, no human disease has been attributed to defects in the genes coding for ALDRP (ABCD2), PMP70 (ABCD3) or PMP69 (ABCD4).”
The paper relies on this without support.
The functions of ABCD2, ABCD3 and ABCD4 in higher eukaryotes are unknown.
“The functions of the three other transporters in higher eukaryotes are unknown”
The paper relies on this without support.
No human syndrome compatible with the abcd2-/- phenotype has been linked to the ABCD2 locus at 12q1.1-1.2.
“To date, no human syndrome compatible with this phenotype has been linked to the ABCD2 locus at 12q1.1 -1.2.”
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 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 [196]
Experimental — accuracy not yet measuredThe cited source says what the claim attributes to it.
In the citing paper
“In Abcd2-deficient mice, saturated VLCFA do not generally accumulate; increased C26∶0 levels have been detected exclusively in the dorsal root ganglia of young Abcd2-deficient mice [33].”
From Abcd2 Is a Strong Modifier of the Metabolic Impairments in Peritoneal Macrophages of Abcd1-Deficient Mice (2014) · cites this paper as [33]
The cited source says what the claim attributes to it.
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 [33]
The cited source says what the claim attributes to it.
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 [196]
The cited source says what the claim attributes to it.
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 [196]
The cited source says what the claim attributes to it.
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 [196]
The cited source says what the claim attributes to it.
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 [196]
The cited source says what the claim attributes to it.
In the citing paper
“Moreover, Acbd2–/– mice develop signs of ataxia corresponding to a significant decline in cerebellar Purkinje neurons at the age of 20 months, suggesting that the more severe locomotor phenotype is associated with the degeneration of central motor centers (Ferrer et al., 2005).”
From The neurological pathology of peroxisomal ACBD5 deficiency – lessons from patients and mouse models (2025) · cites this paper as Ferrer et al., 2005
The cited source says what the claim attributes to it.
In the citing paper
“Moreover, Acbd2–/– mice develop signs of ataxia corresponding to a significant decline in cerebellar Purkinje neurons at the age of 20 months, suggesting that the more severe locomotor phenotype is associated with the degeneration of central motor centers (Ferrer et al., 2005).”
From The neurological pathology of peroxisomal ACBD5 deficiency – lessons from patients and mouse models (2025) · cites this paper as Ferrer et al., 2005
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
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Biochemical function of ALDRP
“In this cell type, lack of ALDRP is neither translated into an accumulation of VLCFAs nor into a decrease of C26:0 b-oxidation capacity (Fig. 3G and H).”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Immunohistochemistry
“(F) Capacity of adult fibroblasts to b-oxidize C26:0, expressed as picomoles per hour per milligram of protein.”
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
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Biochemical function of ALDRP
“A clear reduction of non-phosphorylated neurofilaments in Purkinje cells (SMI 32 antibody) (23) indicated reduced dendritic arborization (Fig. 7E and F).”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Immunohistochemistry
“(F) Focal upregulations of GFAP expression in aldr2/2.(H) Strong reduction of non-phosphorylated neurofilaments in Purkinje cells was seen by staining with anti-SMI32.”
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
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Biochemical function of ALDRP
“We could indeed detect an accumulation of C26:0 in DRGs from abcd22/2 mice (Fig. 3I and J, P , 0.002).”
§ Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage > Immunohistochemistry
“(G and H) Quantification of saturated VLCFA levels by ESI-MS in adult fibroblasts and DRG samples, expressed as micromoles per milligram of protein.”
Citations
Cites
Peroxisomes (microbodies and related particles).
De Duve, C., & Baudhuin, P. (1966) · Physiological Reviews · doi:10.1152/physrev.1966.46.2.323 · Cited by 1,666
Putative X-linked adrenoleukodystrophy gene shares unexpected homology with ABC transporters
Mosser, J., Douar, A.-M., Sarde, C.-O., Kioschis, P., Feil, R., Moser, H., Poustka, A.-M., Mandel, J.-L., & Aubourg, P. (1993) · Nature · doi:10.1038/361726a0 · Cited by 1,202
Self-citationSternberger, L. A., & Sternberger, N. H. (1983) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.80.19.6126 · Cited by 1,158
Hyde, S. C., Emsley, P., Hartshorn, M. J., Mimmack, M. M., Gileadi, U., Pearce, S. R., Gallagher, M. P., Gill, D. R., Hubbard, R. E., & Higgins, C. F. (1990) · Nature · doi:10.1038/346362a0 · Cited by 1,145
Disruption of Cnp1 uncouples oligodendroglial functions in axonal support and myelination
Lappe-Siefke, C., Goebbels, S., Gravel, M., Nicksch, E., Lee, J., Braun, P. E., Griffiths, I. R., & Nave, K.-A. (2003) · Nature Genetics · doi:10.1038/ng1095 · Cited by 1,036
Bjartmar, C., Wujek, J. R., & Trapp, B. D. (2003) · Journal of the Neurological Sciences · doi:10.1016/s0022-510x(02)00069-2 · Cited by 573
Qualitative alteration in substrate specificity of mitochondrial monoamine oxidase in brain
Moskvitina, T. A., Kamyshanskaya, N. S., Kaverina, L. P., & Gorkin, V. Z. (1976) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.1976.tb04462.x · Cited by 440
Peroxisome biogenesis disorders
Gould, S. J., & Valle, D. L. (2000) · Trends in Genetics · doi:10.1016/s0168-9525(00)02056-4 · Cited by 354
Fan, C.-Y., Pan, J., Usuda, N., Yeldandi, A. V., Rao, M. S., & Reddy, J. K. (1998) · Journal of Biological Chemistry · doi:10.1074/jbc.273.25.15639 · Cited by 343
Edgar, J. M., McLaughlin, M., Yool, D., Zhang, S.-C., Fowler, J. H., Montague, P., Barrie, J. A., McCulloch, M. C., Duncan, I. D., Garbern, J., et al. (2004) · The Journal of Cell Biology · doi:10.1083/jcb.200312012 · Cited by 328
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. Supplement to Vol. 73, p. 199.
Cited by
Sorted by citations
The Peroxisome-Mitochondria Connection: How and Why?
Fransen, M., Lismont, C., & Walton, P. (2017) · International Journal of Molecular Sciences · doi:10.3390/ijms18061126 · Cited by 351
Biochemistry and genetics of inherited disorders of peroxisomal fatty acid metabolism
Veldhoven, P. P. V. (2010) · Journal of Lipid Research · doi:10.1194/jlr.r005959 · Cited by 341
Redox interplay between mitochondria and peroxisomes
Lismont, C., Nordgren, M., Van Veldhoven, P. P., & Fransen, M. (2015) · Frontiers in Cell and Developmental Biology · doi:10.3389/fcell.2015.00035 · Cited by 214
Early oxidative damage underlying neurodegeneration in X-adrenoleukodystrophy
Fourcade, S., Lopez-Erauskin, J., Galino, J., Duval, C., Naudi, A., Jove, M., Kemp, S., Villarroya, F., Ferrer, I., Pamplona, R., et al. (2008) · Human Molecular Genetics · doi:10.1093/hmg/ddn085 · Cited by 214
“5 and 17 months of age, after perfusion with PFA4% basically as described (5,52).”
BackgroundMethodSelf-citationPeroxisome‐mitochondria interplay and disease
Schrader, M., Costello, J. L., Godinho, L. F., & Islinger, M. (2015) · Journal of Inherited Metabolic Disease · doi:10.1007/s10545-015-9819-7 · Cited by 232
Metadata
- Authors
- Ferrer, I., Kapfhammer, J. P., Hindelang, C., Kemp, S., Troffer-Charlier, N., Broccoli, V., Callyzot, N., Mooyer, P., Selhorst, J., Vreken, P., Wanders, R. J. A., Mandel, J. L., & Pujol, A.
- Affiliations
- University of Basel (CH)
- Centre National de la Recherche Scientifique (FR)
- Amsterdam UMC Location University of Amsterdam (NL)
- Sig Holding (Switzerland) (CH)
- Institució Catalana de Recerca i Estudis Avançats (ES)
- Venue
- Human Molecular Genetics
- Publisher
- Oxford University Press (OUP)
- Published
- 2005-10-13
- Volume
- 14
- Issue
- 23
- Pages
- 3565-3577
- ISSN
- 0964-6906 (ISSN-L)
- 1460-2083
- OpenAlex
- W2099862428
- Cited by
- 106
- References
- 56
- Citations by year
- 2025: 3 · 2024: 1 · 2023: 5 · 2022: 5 · 2021: 4 · 2020: 2 · 2019: 2 · 2018: 7 · 2017: 4 · 2016: 2 · 2015: 6 · 2014: 7 · 2013: 9 · 2012: 5
- FWCI
- 2.56
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Human Molecular Genetics (publishedVersion)
- PubMed (publishedVersion)
- reroDoc Digital Library (submittedVersion)
- Data Archiving and Networked Services (DANS) (submittedVersion)
- HAL (Le Centre pour la Communication Scientifique Directe) (submittedVersion)
- Pure Amsterdam UMC (submittedVersion)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Peroxisome Proliferator-Activated Receptors
- Health Sciences › Medicine › Physiology (medical) › Adipose Tissue and Metabolism
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
- Keywords
- ABCD2, peroxisomal ABC transporters, late-onset ataxia, very long chain fatty acids, Purkinje cells, axonal degeneration, mitochondrial damage, endoplasmic reticulum damage
- MeSH terms
- ATP Binding Cassette Transporter, Subfamily D, Animals, Behavior, Animal, Cerebellum, Disease Models, Animal, Endoplasmic Reticulum, Golgi Apparatus, Mitochondria, Spinal Cord
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