The DOI matches its Crossref record; 66 of the 67 references it cites were found in a registry or an index.
Expression of the Adrenoleukodystrophy Protein in the Human and Mouse Central Nervous System
Open accessAbstract
The gene mutated in X-linked adrenoleukodystrophy (ALD), a progressive demyelinating disease, codes for a protein (ALDP) involved in very-long-chain fatty acid (VLCFA) transport. The expression of ALDP and of two peroxisomal enzymes involved in beta-oxidation of VLCFA, acyl-CoA oxidase, and catalase was studied in human and mouse brain. The pattern of expression was similar in both species. While acyl-CoA oxidase and catalase are found in all types of CNS cells, including neurons and oligodendrocytes, ALDP expression is restricted mostly to the white matter and endothelial cells. ALDP is highly expressed in astrocytes and microglial cells in vivo and in regenerating oligodendrocytes in vitro. In contrast, in vivo, ALDP is detected in much fewer oligodendrocytes and quantitative Western blot analysis confirmed the lower abundance of ALDP in these cells than in astrocytes. Only oligodendrocytes localized in corpus callosum, internal capsules, and anterior commissure express ALDP at levels comparable to those seen in astrocytes. In ALD, demyelination is first detected in these white matter regions, suggesting that the ALD gene mutation selectively affects those oligodendrocytes strongly expressing ALDP. Because of their failure to express ALDP, microglia and astrocytes may also contribute to demyelination in ALD patients.
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In the citing paper
“ALDP expression varied in other OL subpopulations, weak in subcortical white matter, cerebellum, brainstem, and pons, and absent in cerebral cortex or cerebellar neurons.”
From Revisiting the Pathogenesis of X-Linked Adrenoleukodystrophy (2025) · cites this paper as [156]
In the citing paper
“P. Aubourg’s team found ALDP to be highly expressed in the brains of four infants who died from non-metabolic pathologies: in astrocytes, microglia, perivascular macrophages, endothelial cells, and in a subset of OLs located in the corpus callosum, internal capsules, and anterior commissure [156], the regions where demyelination is first seen on MR images of cALD.”
From Revisiting the Pathogenesis of X-Linked Adrenoleukodystrophy (2025) · cites this paper as [156]
In the citing paper
“In contrast, two peroxisomal enzymes involved in β-oxidation of VLCFA, acyl-CoA oxidase, and catalase were expressed in all human and mouse brain cells, including neurons and endothelial cells [156].”
From Revisiting the Pathogenesis of X-Linked Adrenoleukodystrophy (2025) · cites this paper as [156]
In the citing paper
“In human X-ALD brains, VLCFA accumulation occurs mostly in astrocytes, microglia, endothelial cells, and OLs located in the corpus callosum, internal capsules, and anterior commissure [156].”
From Revisiting the Pathogenesis of X-Linked Adrenoleukodystrophy (2025) · cites this paper as [156]
In the citing paper
“Their peroxisome content is abundant [166,253,315], and they express abundant ALDP in normal microglia in human and mouse brains [156].”
From Revisiting the Pathogenesis of X-Linked Adrenoleukodystrophy (2025) · cites this paper as [156]
In the citing paper
“As expected, no detectable ALDP was present in astrocytes, OLs, or microglia [156]”
From Revisiting the Pathogenesis of X-Linked Adrenoleukodystrophy (2025) · cites this paper as [156]
In the citing paper
“They abundantly express ALDP [156,157,416].”
From Revisiting the Pathogenesis of X-Linked Adrenoleukodystrophy (2025) · cites this paper as [156]
In the citing paper
“In the brain, peroxisomes appear as electron-dense single membrane-bound organelles that have been detected in all neural cell types, namely in neurons [13], oligodendrocytes [14,15] and astrocytes [13] and microglia and endothelial cells [16].”
From Peroxisomes in brain development and function (2016) · cites this paper as [16]
In the citing paper
“In the human brain, based on immunohistochemical detection, ABCD1 is predominantly expressed in oligodendrocytes, microglia, astrocytes and endothelial cells but not in most neurons, with the exception of a few regions: hypothalamus, basal nucleus of Meynert, periaqueductal gray matter and the locus coeruleus [16,30].”
From Peroxisomes in brain development and function (2016) · cites this paper as [16]
In the citing paper
“In the CNS, ALDP is mostly expressed in oligodendrocytes, microglia, astrocytes and endothelial cells [84], but not in most neurons with the exception of hypothalamus, basal nucleus of Meynert, periaqueductal gray matter and in the locus coeruleus [85].”
From X-linked adrenoleukodystrophy: Clinical, metabolic, genetic and pathophysiological aspects (2012) · cites this paper as [84]
Citations
Cites
Bradford, M. M. (1976) · Analytical Biochemistry · doi:10.1016/0003-2697(76)90527-3 · Cited by 255,302
The Metabolic and Molecular Bases of Inherited Disease
Scriver, R. C. (1995) · Medical Entomology and Zoology · Cited by 12,101
ABC Transporters: From Microorganisms to Man
Higgins, C. F. (1992) · Annual Review of Cell Biology · doi:10.1146/annurev.cb.08.110192.000435 · Cited by 3,985
Perivascular microglial cells of the CNS are bone marrow-derived and present antigen in vivo
Hickey, W. F., & Kimura, H. (1988) · Science · doi:10.1126/science.3276004 · Cited by 1,466
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-citationRanscht, B., Clapshaw, P. A., Price, J., Noble, M., & Seifert, W. E. (1982) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.79.8.2709 · Cited by 834
Cytokines and the nervous system I: expression and recognition
Hopkins, S. J., & Rothwell, N. J. (1995) · Trends in Neurosciences · doi:10.1016/0166-2236(95)80029-2 · Cited by 793
Microglial cell cytotoxicity of oligodendrocytes is mediated through nitric oxide.
Merrill, J. E., Ignarro, L. J., Sherman, M. P., Melinek, J., & Lane, T. E. (1993) · The Journal of Immunology · doi:10.4049/jimmunol.151.4.2132 · Cited by 782
The oligodendrocyte and its many cellular processes
PFEIFFER, S., WARRINGTON, A., & BANSAL, R. (1993) · Trends in Cell Biology · doi:10.1016/0962-8924(93)90213-k · Cited by 737
Adrenoleukodystrophy: A Clinical and Pathological Study of 17 Cases
Schaumburg, H. H. (1975) · Archives of Neurology · doi:10.1001/archneur.1975.00490510033001 · Cited by 475
Research works printed but not linked: 1
- Dickson, 1986, Multinucleated giant cells in AIDS, origin from endogenous microglia?, Arch. Pathol. Lab. Med., 110, 967
Cited by
Sorted by citations
Concepts of myelin and myelination in neuroradiology.
Barkovich, A. J. (2000) · PubMed Central · Cited by 460
Peroxide detoxification by brain cells
Dringen, R., Pawlowski, P. G., & Hirrlinger, J. (2004) · Journal of Neuroscience Research · doi:10.1002/jnr.20280 · Cited by 455
X-linked adrenoleukodystrophy: Clinical, metabolic, genetic and pathophysiological aspects
Kemp, S., Berger, J., & Aubourg, P. (2012) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2012.03.012 · Cited by 283
Self-citationBrüstle, O., Choudhary, K., Karram, K., Huttner, A. J., Murray, K., Dubois-Dalcq, M. E., & McKay, R. D. G. (1998) · Nature Biotechnology · doi:10.1038/3481 · Cited by 299
Self-citationOxidative and Antioxidative Potential of Brain Microglial Cells
Dringen, R. (2005) · Antioxidants and Redox Signaling · doi:10.1089/ars.2005.7.1223 · Cited by 270
Gene redundancy and pharmacological gene therapy: Implications for X-linked adrenoleukodystrophy
Kemp, S., Wei, H.-M., Lu, J.-F., Braiterman, L. T., McGuinness, M. C., Moser, A. B., Watkins, P. A., & Smith, K. D. (1998) · Nature Medicine · doi:10.1038/3242 · Cited by 249
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
Role 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
The physiological functions of human peroxisomes
Wanders, R. J. A., Baes, M., Ribeiro, D., Ferdinandusse, S., & Waterham, H. R. (2023) · Physiological Reviews · doi:10.1152/physrev.00051.2021 · Cited by 228
Pujol, A. (2002) · Human Molecular Genetics · doi:10.1093/hmg/11.5.499 · Cited by 214
X-linked adrenoleukodystrophy: Clinical, biochemical and pathogenetic aspects
Berger, J., & Gärtner, J. (2006) · Biochimica et Biophysica Acta (BBA) - Molecular Cell Research · doi:10.1016/j.bbamcr.2006.07.010 · Cited by 199
Peroxisomes in brain development and function
Berger, J., Dorninger, F., Forss-Petter, S., & Kunze, M. (2016) · Biochimica et Biophysica Acta (BBA) - Molecular Cell Research · doi:10.1016/j.bbamcr.2015.12.005 · Cited by 194
“types, namely in neurons [13], oligodendrocytes [14,15] and astrocytes [13] and microglia and endothelial cells [16].”
BackgroundX-linked adrenoleukodystrophy: Genes, mutations, and phenotypes
Smith, K. D., Kemp, S., Braiterman, L. T., Lu, J.-F., Wei, H.-M., Geraghty, M., Stetten, G., Bergin, J. S., Pevsner, J., & Watkins, P. A. (1999) · Neurochemical Research · doi:10.1023/a:1022535930009 · Cited by 190
Hein, S., Schonfeld, P., Kahlert, S., & Reiser, G. (2008) · Human Molecular Genetics · doi:10.1093/hmg/ddn066 · Cited by 180
Human CD34 + cells differentiate into microglia and express recombinant therapeutic protein
Asheuer, M., Pflumio, F., Benhamida, S., Dubart‐Kupperschmitt, A., Fouquet, F., Imai, Y., Aubourg, P. R., & Cartier, N. (2004) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0306431101 · Cited by 163
Self-citationNetik, A., Forss-Petter, S., Holzinger, A., Molzer, B., Unterrainer, G., & Berger, J. (1999) · Human Molecular Genetics · doi:10.1093/hmg/8.5.907 · Cited by 148
Role of ALDP (ABCD1) and Mitochondria in X-Linked Adrenoleukodystrophy
McGuinness, M. C., Lu, J.-F., Zhang, H.-P., Dong, G.-X., Heinzer, A. K., Watkins, P. A., Powers, J., & Smith, K. D. (2003) · Molecular and Cellular Biology · doi:10.1128/mcb.23.2.744-753.2003 · Cited by 147
Homo- and Heterodimerization of Peroxisomal ATP-binding Cassette Half-transporters
Liu, L. X., Janvier, K., Berteaux-Lecellier, V., Cartier, N., Benarous, R., & Aubourg, P. (1999) · Journal of Biological Chemistry · doi:10.1074/jbc.274.46.32738 · Cited by 141
Self-citationGeneral aspects and neuropathology of X-linked adrenoleukodystrophy
Ferrer, I., Aubourg, P., & Pujol, A. (2010) · Brain Pathology · doi:10.1111/j.1750-3639.2010.00390.x · Cited by 139
Self-citationThe neurobiology of X-linked adrenoleukodystrophy, a demyelinating peroxisomal disorder
Dubois-Dalcq, M., Feigenbaum, V., & Aubourg, P. (1999) · Trends in Neurosciences · doi:10.1016/s0166-2236(98)01319-8 · Cited by 121
Self-citationABC Transporters in the CNS – An Inventory
Hartz, A. M. S., & Bauer, B. (2011) · Current Pharmaceutical Biotechnology · doi:10.2174/138920111795164020 · Cited by 124
Theodoulou, F. L., Holdsworth, M. J., & Baker, A. (2006) · FEBS Letters · doi:10.1016/j.febslet.2005.12.095 · Cited by 119
Biochemical aspects of X-linked adrenoleukodystrophy
Kemp, S., & Wanders, R. (2010) · Brain Pathology · doi:10.1111/j.1750-3639.2010.00391.x · Cited by 116
Kemp, S., Theodoulou, F. L., & Wanders, R. J. (2011) · British Journal of Pharmacology · doi:10.1111/j.1476-5381.2011.01435.x · Cited by 115
Berger, J., Albet, S., Bentejac, M., Netik, A., Holzinger, A., Roscher, A. A., Bugaut, M., & Forss‐Petter, S. (1999) · European Journal of Biochemistry · doi:10.1046/j.1432-1327.1999.00772.x · Cited by 106
Braiterman, L. (1998) · Human Molecular Genetics · doi:10.1093/hmg/7.2.239 · Cited by 98
ABC Transporters Are Key Players in Alzheimer’s Disease
Pereira, C. D., Martins, F., Wiltfang, J., Silva, O. A. B. D. C. E., & Rebelo, S. (2017) · Journal of Alzheimer s Disease · doi:10.3233/jad-170639 · Cited by 94
Troffer-Charlier, N., Doerflinger, N., Metzger, E., Fouquet, F., Mandel, J.-L., & Aubourg, P. (1998) · European Journal of Cell Biology · doi:10.1016/s0171-9335(98)80121-0 · Cited by 85
Self-citationCristiano, L., Bernardo, A., & Ceru`, M. P. (2001) · Journal of Neurocytology · doi:10.1023/a:1016525716209 · Cited by 81
Behl, T., Kaur, I., Sehgal, A., Kumar, A., Uddin, M. S., & BUNGAU, S. G. (2020) · Molecular Neurobiology · doi:10.1007/s12035-020-02211-x · Cited by 78
Adenoassociated virus serotype 9-mediated gene therapy for x-linked adrenoleukodystrophy
Gong, Y., Mu, D., Prabhakar, S., Moser, A., Musolino, P., Ren, J., Breakefield, X. O., Maguire, C. A., & Eichler, F. S. (2015) · Molecular Therapy · doi:10.1038/mt.2015.6 · Cited by 74
Höftberger, R., Kunze, M., Weinhofer, I., Aboul-Enein, F., Voigtländer, T., Oezen, I., Amann, G., Bernheimer, H., Budka, H., & Berger, J. (2007) · Neurobiology of Disease · doi:10.1016/j.nbd.2007.07.007 · Cited by 69
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Lee, J. Y., & Petratos, S. (2016) · Molecular Neurobiology · doi:10.1007/s12035-016-0013-1 · Cited by 71
Peroxisomes, myelination, and axonal integrity in the CNS
Baes, M., & Aubourg, P. (2009) · The Neuroscientist · doi:10.1177/1073858409336297 · Cited by 65
Self-citationDepreter, M., Espeel, M., & Roels, F. (2003) · Microscopy Research and Technique · doi:10.1002/jemt.10330 · Cited by 63
Benhamida, S., Pflumio, F., Dubart-Kupperschmitt, A., Zhao-Emonet, J.-C., Cavazzana-Calvo, M., Rocchiccioli, F., Fichelson, S., Aubourg, P., Charneau, P., & Cartier, N. (2003) · Molecular Therapy · doi:10.1016/s1525-0016(03)00002-9 · Cited by 61
“The ALD protein is expressed in glial cells [3], including microglia, and belongs to the superfamily of ATPbinding cassette transporters [4].”
BackgroundSelf-citationPeroxisomal Metabolite and Cofactor Transport in Humans
Chornyi, S., IJlst, L., van Roermund, C. W. T., Wanders, R. J. A., & Waterham, H. R. (2021) · Frontiers in Cell and Developmental Biology · doi:10.3389/fcell.2020.613892 · Cited by 64
Mouse liver PMP70 and ALDP: homomeric interactions prevail in vivo
Guimarães, C. P., Domingues, P. M., Aubourg, P. R., Fouquet, F., Pujol, A., Jiménez‐Sánchez, G., Sá-Miranda, C., & Azevedo, J. E. (2004) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2004.04.001 · Cited by 60
Self-citationMouse Models of Myelin Diseases
Werner, H. B., Jung, M., Klugmann, M., Sereda, M. W., Griffiths, I. R., & Nave, K. (1998) · Brain Pathology · doi:10.1111/j.1750-3639.1998.tb00200.x · Cited by 57
Kruska, N., Schönfeld, P., Pujol, A., & Reiser, G. (2015) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2015.01.005 · Cited by 56
Castellano, A., Papinutto, N., Cadioli, M., Brugnara, G., Iadanza, A., Scigliuolo, G. M., Pareyson, D., Uziel, G., Köhler, W., Aubourg, P. R., et al. (2016) · Brain · doi:10.1093/brain/aww068 · Cited by 55
Self-citationMicroglial dysfunction as a key pathological change in adrenomyeloneuropathy
Gong, Y., Sasidharan, N., Laheji, F., Frosch, M., Musolino, P., Tanzi, R., Kim, D. Y., Biffi, A., El Khoury, J., & Eichler, F. (2017) · Annals of Neurology · doi:10.1002/ana.25085 · Cited by 54
A microglial cell model for acyl-CoA oxidase 1 deficiency
Raas, Q., Saih, F.-E., Gondcaille, C., Trompier, D., Hamon, Y., Leoni, V., Caccia, C., Nasser, B., Jadot, M., Ménétrier, F., et al. (2018) · Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids · doi:10.1016/j.bbalip.2018.10.005 · Cited by 57
Genin, E. C., Geillon, F., Gondcaille, C., Athias, A., Gambert, P., Trompier, D., & Savary, S. (2011) · Journal of Biological Chemistry · doi:10.1074/jbc.m110.211912 · Cited by 53
Characterization of the human ω‐oxidation pathway for ω‐hydroxy‐very‐long‐chain fatty acids
Sanders, R., Ofman, R., Dacremont, G., Wanders, R. J. A., & Kemp, S. (2008) · The FASEB Journal · doi:10.1096/fj.07-099150 · Cited by 51
Baarine, M., Ragot, K., Genin, E. C., Hajj, H. I. E., Trompier, D., Andreoletti, P., Ghandour, M. S., Ménétrier, F., Cherkaoui‐Malki, M., Savary, S., et al. (2009) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.2009.06311.x · Cited by 57
Flavigny, E., Sanhaj, A., Aubourg, P. R., & Cartier, N. (1999) · FEBS Letters · doi:10.1016/s0014-5793(99)00379-8 · Cited by 49
Self-citationABCD1 dysfunction alters white matter microvascular perfusion
Lauer, A., Da, X., Hansen, M. B., Boulouis, G., Ou, Y., Cai, X., Liberato Celso Pedrotti, A., Kalpathy-Cramer, J., Caruso, P., Hayden, D. L., et al. (2017) · Brain · doi:10.1093/brain/awx262 · Cited by 48
Two Novel Epilepsy-Linked Mutations Leading to a Loss of Function of LGI1
Chabrol, E., Popescu, C., Gourfinkel‐An, I., Trouillard, O., Depienne, C., Senechal, K. R., Baulac, M., Leguern, É., & Baulac, S. (2007) · Archives of Neurology · doi:10.1001/archneur.64.2.217 · Cited by 47
Peroxisomal Disorders: A Review on Cerebellar Pathologies
Munter, S. D., Verheijden, S., Régal, L., & Baes, M. I. (2015) · Brain Pathology · doi:10.1111/bpa.12290 · Cited by 46
Baarine, M., Ragot, K., Athias, A., Nury, T., Kattan, Z., Genin, E. C., Andreoletti, P., Ménétrier, F., Riedinger, J.-M., Bardou, M., et al. (2012) · NeuroToxicology · doi:10.1016/j.neuro.2011.10.007 · Cited by 43
Aubourg, P. R., & Dubois-Dalcq, M. E. (2000) · Glia · doi:10.1002/(sici)1098-1136(20000115)29:2 · Cited by 42
Self-citationDiffusion tensor–based imaging reveals occult abnormalities in adrenomyeloneuropathy
Dubey, P., Fatemi, A., Huang, H., Nagae‐Poetscher, L., Wakana, S., Barker, P. B., van Zijl, P., Moser, H. W., Mori, S., & Raymond, G. V. (2005) · Annals of Neurology · doi:10.1002/ana.20643 · Cited by 40
Retroviral Transfer and Long-Term Expression of the Adrenoleukodystrophy Gene in Human CD34 + Cells
Doerflinger, N., Micléa, J.-M., Lopez, J., Chomienne, C., Bougneres, P. F., Aubourg, P. R., & Cartier, N. (1998) · Human Gene Therapy · doi:10.1089/hum.1998.9.7-1025 · Cited by 39
Self-citationImmunocytochemical localization of acyl-CoA oxidase in the rat central nervous system
Vecchioli, S. F., Moreno, S., & Cerù, M. P. (2001) · Journal of Neurocytology · doi:10.1023/a:1011913223541 · Cited by 38
Seven-Tesla proton magnetic resonance spectroscopic imaging in adult X-linked adrenoleukodystrophy
Ratai, E., Kok, T., Wiggins, C., Wiggins, G., Grant, E., Gagoski, B., O’Neill, G., Adalsteinsson, E., & Eichler, F. (2008) · Archives of Neurology · doi:10.1001/archneur.65.11.1488 · Cited by 37
Apoptosis in the Central Nervous System of Cerebral Adrenoleukodystrophy Patients
Feigenbaum, V., Gélot, A., Casanova, P., Daumas‐Duport, C., Aubourg, P. R., & Dubois-Dalcq, M. E. (2000) · Neurobiology of Disease · doi:10.1006/nbdi.2000.0315 · Cited by 37
Self-citationEmerging Role of ABC Transporters in Glia Cells in Health and Diseases of the Central Nervous System
Villa, M., Wu, J., Hansen, S. M., & Pahnke, J. (2024) · Cells · doi:10.3390/cells13090740 · Cited by 43
Presence and inducibility of peroxisomes in a human glioblastoma cell line
Cimini, A., Cristiano, L., Bernardo, A., Vecchioli, S. F., Stefanini, S., & Cerù, M. P. (2000) · Biochimica et Biophysica Acta (BBA) - General Subjects · doi:10.1016/s0304-4165(00)00036-2 · Cited by 31
Unterrainer, G. (2000) · Human Molecular Genetics · doi:10.1093/hmg/9.18.2609 · Cited by 30
Toublanc, E., Benraiss, A., Bonnin, D., Blouin, V., Brument, N., Cartier, N., Epstein, A. L., Moullier, P., & Salvetti, A. (2004) · The Journal of Gene Medicine · doi:10.1002/jgm.542 · Cited by 29
ABCD1 translation–initiator mutation demonstrates genotype–phenotype correlation for AMN
O’Neill, G. N., Aoki, M., & Brown, R. H. (2001) · Neurology · doi:10.1212/wnl.57.11.1956 · Cited by 28
Lauer, A., Speroni, S. L., Choi, M., Da, X., Duncan, C., McCarthy, S., Krishnan, V., Lusk, C. A., Rohde, D., Hansen, M. B., et al. (2023) · Nature Communications · doi:10.1038/s41467-023-37262-w · Cited by 26
Structure-Function Analysis of Peroxisomal ATP-binding Cassette Transporters Using Chimeric Dimers
Geillon, F., Gondcaille, C., Charbonnier, S., Van Roermund, C. W., Lopez, T. E., Dias, A. M. M., Pais de Barros, J.-P., Arnould, C., Wanders, R. J., Trompier, D., et al. (2014) · Journal of Biological Chemistry · doi:10.1074/jbc.m114.575506 · Cited by 24
Bergner, C. G., Genc, N., Hametner, S., Franz, J., van der Meer, F., Mitkovski, M., Weber, M. S., Stoltenburg‐Didinger, G., Kühl, J., Köhler, W., et al. (2021) · Glia · doi:10.1002/glia.24042 · Cited by 23
Nagase, T., Shimozawa, N., Takemoto, Y., Suzuki, Y., Komori, M., & Kondo, N. (2004) · Biochimica et Biophysica Acta (BBA) - General Subjects · doi:10.1016/j.bbagen.2004.01.004 · Cited by 22
Lannuzel, A., Aubourg, P., & Tardieu, M. (1998) · European journal of paediatric neurology · doi:10.1016/1090-3798(98)01002-7 · Cited by 15
Self-citationProfiling and Imaging of Phospholipids in Brains of Abcd1‐Deficient Mice
Hama, K., Fujiwara, Y., Morita, M., Yamazaki, F., Nakashima, Y., Takei, S., Takashima, S., Setou, M., Shimozawa, N., Imanaka, T., et al. (2018) · Lipids · doi:10.1002/lipd.12022 · Cited by 22
Wang, R., Monuki, E. S., Powers, J., Schwartz, P. H., Watkins, P. A., Shi, Y., Moser, A. B., Shrier, D. A., Waterham, H. R., Nugent, D. J., et al. (2014) · Journal of Inherited Metabolic Disease · doi:10.1007/s10545-014-9698-3 · Cited by 22
Morita, M., Shinbo, S., 朝日彰子, & Imanaka, T. (2012) · Biological and Pharmaceutical Bulletin · doi:10.1248/bpb.b12-00411 · Cited by 22
Advances in AAV-mediated gene replacement therapy for pediatric monogenic neurological disorders
Zhou, L., Wang, Y., Xu, Y., Zhang, Y., & Zhu, C. (2024) · Molecular Therapy — Methods & Clinical Development · doi:10.1016/j.omtm.2024.101357 · Cited by 22
(2006) · Springer eBooks · doi:10.1007/3-540-29623-9_9160 · Cited by 21
The pathology of X-linked adrenoleukodystrophy: Tissue specific changes as a clue to pathophysiology
Yska, H. A. F., Engelen, M., & Bugiani, M. (2024) · Orphanet Journal of Rare Diseases · doi:10.1186/s13023-024-03105-0 · Cited by 20
“Our knowledge on the physical ABCD1-distribution in the CNS is mostly based on studies by Fouquet et al. and Höftberger et al. [36, 37].”
BackgroundMethodGenotype and Protein Expression After Bone Marrow Transplantation for Adrenoleukodystrophy
Schönberger, S., Roerig, P., Schneider, D. T., Reifenberger, G., Göbel, U., & Gärtner, J. (2007) · Archives of Neurology · doi:10.1001/archneur.64.5.noc60105 · Cited by 19
Trompier, D., & Savary, S. (2013) · Colloquium Series on The Genetic Basis of Human Disease · doi:10.4199/c00075ed1v01y201303gbd004 · Cited by 17
Żuberek, M., Stępkowski, T. M., Kruszewski, M. K., & Grzelak, A. (2018) · Mechanisms of Ageing and Development · doi:10.1016/j.mad.2018.02.004 · Cited by 16
Santos, A., Borges, N., Cerejo, A., Sarmento, A., & Azevedo, I. (2005) · Neurochemical Research · doi:10.1007/s11064-005-2750-9 · Cited by 15
Song, S., Kato, C., Adachi, E., Moriya‐Sato, A., Ogashiwa, M., Umeda, R., & Hashimoto, N. (2007) · Journal of Neuroscience Research · doi:10.1002/jnr.21456 · Cited by 14
On the Front of X-Linked Adrenoleukodystrophy
Aubourg, P. R. (1999) · Neurochemical Research · doi:10.1023/a:1022583813171 · Cited by 12
Self-citationRevisiting the Pathogenesis of X-Linked Adrenoleukodystrophy
Bougnères, P., & Le Stunff, C. (2025) · Genes · doi:10.3390/genes16050590 · Cited by 11
Differential activities of peroxisomes along the mouse intestinal epithelium
Morvay, P. L., Baes, M. I., & Veldhoven, P. P. V. (2017) · Cell Biochemistry and Function · doi:10.1002/cbf.3255 · Cited by 11
Debbabi, M., Nury, T., Helali, I., Karym, E. M., Geillon, F., Gondcaille, C., Trompier, D., Najid, A., Terreau, S., Bezine, M., et al. (2017) · Methods in molecular biology · doi:10.1007/978-1-4939-6937-1_25 · Cited by 11
Role of Basal Forebrain Neurons in Adrenomyeloneuropathy in Mice and Humans
Gong, Y., Laheji, F., Berenson, A., Li, Y., Moser, A., Qian, A., Frosch, M., Sadjadi, R., Hahn, R., Maguire, C. A., et al. (2024) · Annals of Neurology · doi:10.1002/ana.26849 · Cited by 11
Hu, Y., Gu, J., Jin, X., Wu, X., Li, H., Bai, L., Wu, J., & Li, X. (2024) · Neuropharmacology · doi:10.1016/j.neuropharm.2024.110208 · Cited by 9
Martinović, K., Bauer, J., Kunze, M., Berger, J., & Forss‐Petter, S. (2023) · Acta Neuropathologica Communications · doi:10.1186/s40478-023-01595-w · Cited by 10
Holzinger, A., Muntau, A. C., Mayerhofer, P., Kammerer, S., Albet, S., Bugaut, M., & Roscher, A. A. (1998) · FEBS Letters · doi:10.1016/s0014-5793(98)00873-4 · Cited by 8
Ohi, T., Takechi, S., Itokazu, N., Shiomi, K., Sugimoto, S., Antoku, Y., Kato, K., Sugimoto, T., Nakayama, T., & Matsukura, S. (2000) · Journal of the Neurological Sciences · doi:10.1016/s0022-510x(00)00349-x · Cited by 7
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Salsano, E. (2015) · Molecular Genetics and Metabolism · doi:10.1016/j.ymgme.2015.02.005 · Cited by 6
The mouse as a model to understand peroxisomal biogenesis and its disorders
Brites, P., Wanders, R. J. A., & Waterham, H. R. (2004) · Drug Discovery Today Disease Models · doi:10.1016/j.ddmod.2004.09.003 · Cited by 6
Noble, M., Mayer‐Pröschel, M., & Miller, R. H. (2006) · Kluwer Academic Publishers-Plenum Publishers eBooks · doi:10.1007/0-387-28117-7_6 · Cited by 6
Mouse Models of Metachromatic Leukodystrophy and Adrenoleukodystrophy
Aubourg, P. R., Sevin, C., & Cartier, N. (2010) · Neuromethods · doi:10.1007/978-1-60761-898-0_26 · Cited by 5
Self-citationPeroxisomes and PPARs in Cultured Neural Cells
Cimini, A., Cristiano, L., Bernardo, A., Benedetti, E., Loreto, S. D., & Cerù, M. P. (2004) · Advances in experimental medicine and biology · doi:10.1007/978-1-4419-9072-3_35 · Cited by 4
Detoxification of hydrogen peroxide by astrocytes
Dringen, R., Liddell, J. R., Knorpp, T., & Robinson, S. R. (2007) · Springer eBooks · doi:10.1007/1-4020-4456-9_4 · Cited by 3
Intracisternal AAV9-MAG- hABCD1 Vector Reverses Motor Deficits in Adult Adrenomyeloneuropathy Mice
Özgür Günes, Y., Le Stunff, C., & Bougnères, P. (2025) · Human Gene Therapy · doi:10.1089/hum.2024.175 · Cited by 2
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Bougneres, P. F., Stunff, C. L., & Badin, R. A. (2025) · Biomedicines · doi:10.3390/biomedicines13081892 · Cited by 1
Antioxidative Defense of Brain Microglial Cells
Dringen, R., & Hirrlinger, J. (2009) · Oxidative stress and disease · doi:10.1201/9781420073522.ch27 · Cited by 1
Peroxisomes during Development and in Distinct Cell Types
Roels, F., Depreter, M., Espeel, M., D'Herde, K. G. M. A., Kerckaert, I., Vamecq, J., & Branden, C. V. D. (2003) · Advances in experimental medicine and biology · doi:10.1007/978-1-4419-9072-3_4 · Cited by 1
Role of Impaired ABC Transporters in Alzheimer’s Disease
Haque, M., Rather, G. A., Siddiqui, E. M., Jahan, S., Khan, A., Makeen, H. A., Alexiou, A. T., & Ashraf, G. M. (2021) · Springer eBooks · doi:10.1007/978-981-16-4558-7_13 · Cited by 1
Myéline et résonance magnétique nucléaire
Tourbah, A., Dormont, D., Galanaud, D., Sedel, F., & Lyon‐Caen, O. (2007) · Revue Neurologique · doi:10.1016/s0035-3787(07)92638-7 · Cited by 1
Aubourg, P. R. (2003) · Steinkopff eBooks · doi:10.1007/978-3-642-57441-2_16 · Cited by 1
Self-citation
Metadata
- Authors
- Fouquet, F., Zhou, J. M., Ralston, E., Murray, K., Troalen, F., Magal, E., Robain, O., Dubois-Dalcq, M., & Aubourg, P.
- Affiliations
- National Institutes of Health (US)
- Institut Pasteur (FR)
- Institut Gustave Roussy (FR)
- Amgen (United States) (US)
- Inserm (FR)
- ORCID
- Ralston, Evelyn
- Venue
- Neurobiology of Disease
- Publisher
- Elsevier BV
- Published
- 1997-01
- Volume
- 3
- Issue
- 4
- Pages
- 271-285
- ISSN
- 0969-9961 (ISSN-L)
- OpenAlex
- W2005093647
- Cited by
- 115
- References
- 67
- Citations by year
- 2026: 1 · 2025: 2 · 2024: 9 · 2023: 5 · 2022: 2 · 2021: 3 · 2020: 1 · 2018: 3 · 2017: 5 · 2016: 2 · 2015: 5 · 2014: 3 · 2013: 2 · 2012: 2
- FWCI
- 5.66
- Type
- journal article
- Language
- en
- Open access
- green
- doaj.org
- Licence
- Locations
- Neurobiology of Disease (publishedVersion)
- PubMed (publishedVersion)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
- Funders
- Ministère de la Santé
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
- Health Sciences › Medicine › Genetics (clinical) › Lysosomal Storage Disorders Research
- Health Sciences › Medicine › Clinical Neurology › Multiple Sclerosis Research Studies
- Keywords
- X-linked adrenoleukodystrophy, very long chain fatty acids, peroxisomal beta-oxidation, acyl-CoA oxidase, catalase, oligodendrocytes, astrocytes, microglia, endothelial cells, white matter demyelination, corpus callosum, internal capsule, anterior commissure
- MeSH terms
- ATP Binding Cassette Transporter, Subfamily D, Member 1, Adult, Animals, Astrocytes, Brain, Catalase, Child, Preschool, Endothelium, Humans, Infant, Newborn, Male, Membrane Proteins, Mice, Inbred C3H, Microbodies, Middle Aged, Nerve Regeneration, Oligodendroglia, Oxidoreductases, Microglia, ATP-Binding Cassette Transporters, Acyl-CoA Oxidase, Mice, Rats
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