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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ALDP expression varies in other oligodendrocyte subpopulations, is weak in subcortical white matter, cerebellum, brainstem, and pons, and is absent in cerebral cortex or cerebellar neurons.
“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]
ALDP is highly expressed in the brains of infants who died from non-metabolic pathologies: in astrocytes, microglia, perivascular macrophages, endothelial cells, and in a subset of oligodendrocytes located in the corpus callosum, internal capsules, and anterior commissure.
“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]
Two peroxisomal enzymes involved in β-oxidation of VLCFA, acyl-CoA oxidase and catalase, are expressed in all human and mouse brain cells, including neurons and endothelial cells.
“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 human X-ALD brains, VLCFA accumulation occurs mostly in astrocytes, microglia, endothelial cells, and oligodendrocytes located in the corpus callosum, internal capsules, and anterior commissure.
“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]
Microglia have abundant peroxisome content and express abundant ALDP in normal microglia in human and mouse brains.
“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]
No detectable ALDP was present in astrocytes, oligodendrocytes, or microglia.
“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]
Endothelial cells abundantly express ALDP.
“They abundantly express ALDP [156,157,416].”
From Revisiting the Pathogenesis of X-Linked Adrenoleukodystrophy (2025) · cites this paper as [156]
In the brain, peroxisomes have been detected in all neural cell types: neurons, oligodendrocytes, astrocytes, microglia and endothelial cells.
“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 human brain, ABCD1 is predominantly expressed in oligodendrocytes, microglia, astrocytes and endothelial cells but not in most neurons, with exceptions including hypothalamus, basal nucleus of Meynert, periaqueductal gray matter and locus coeruleus.
“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 CNS, ALDP is mostly expressed in oligodendrocytes, microglia, astrocytes and endothelial cells, but not in most neurons with the exception of hypothalamus, basal nucleus of Meynert, periaqueductal gray matter and locus coeruleus.
“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]
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Self-citation
- 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 › Physiology › Lysosomal Storage Disorders Research
- Health Sciences › Medicine › Pathology and Forensic Medicine › 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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