Expression of the Adrenoleukodystrophy Protein in the Human and Mouse Central Nervous System
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 β-oxidation of VLCFA, acyl-CoA oxidase, and catalase wa...
| Main Authors: | , , , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Elsevier
1997-01-01
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| Series: | Neurobiology of Disease |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S0969996197901273 |
| _version_ | 1818574102885040128 |
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| author | Francoise Fouquet Jia Min Zhou Evelyn Ralston Kerren Murray Frédéric Troalen Ella Magal Olivier Robain M. Dubois-Dalcq Patrick Aubourg |
| author_facet | Francoise Fouquet Jia Min Zhou Evelyn Ralston Kerren Murray Frédéric Troalen Ella Magal Olivier Robain M. Dubois-Dalcq Patrick Aubourg |
| author_sort | Francoise Fouquet |
| collection | DOAJ |
| description | 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 β-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 cellsin vivoand in regenerating oligodendrocytesin 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. |
| first_indexed | 2024-12-15T00:21:59Z |
| format | Article |
| id | doaj.art-86226d97e6cb4bc894b6577f32e9a5fe |
| institution | Directory Open Access Journal |
| issn | 1095-953X |
| language | English |
| last_indexed | 2024-12-15T00:21:59Z |
| publishDate | 1997-01-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Neurobiology of Disease |
| spelling | doaj.art-86226d97e6cb4bc894b6577f32e9a5fe2022-12-21T22:42:16ZengElsevierNeurobiology of Disease1095-953X1997-01-0134271285Expression of the Adrenoleukodystrophy Protein in the Human and Mouse Central Nervous SystemFrancoise Fouquet0Jia Min Zhou1Evelyn Ralston2Kerren Murray3Frédéric Troalen4Ella Magal5Olivier Robain6M. Dubois-Dalcq7Patrick Aubourg8Unité Inserm U342, Pathologie Métabolique et Hormonale du Developpement, Hôpital St. Vincent de Paul, Paris, France; Unité de Neurovirologie et Régénération du Système Nerveux, Département de Virologie, Institut Pasteur, Paris, France; Laboratory of Viral and Molecular Pathogenesis, National Institutes of Health, Bethesda, Maryland, 20892; Laboratory of Neurobiology, NINDS, National Institutes of Health, Bethesda, Maryland, 20892; Département de Biologie Clinique, Institut Gustave Roussy, Villejuif, France; Amgen, Thousand Oaks, California, 91320; Unité Inserm U29, Hôpital Port-Royal, Paris, FranceUnité Inserm U342, Pathologie Métabolique et Hormonale du Developpement, Hôpital St. Vincent de Paul, Paris, France; Unité de Neurovirologie et Régénération du Système Nerveux, Département de Virologie, Institut Pasteur, Paris, France; Laboratory of Viral and Molecular Pathogenesis, National Institutes of Health, Bethesda, Maryland, 20892; Laboratory of Neurobiology, NINDS, National Institutes of Health, Bethesda, Maryland, 20892; Département de Biologie Clinique, Institut Gustave Roussy, Villejuif, France; Amgen, Thousand Oaks, California, 91320; Unité Inserm U29, Hôpital Port-Royal, Paris, FranceUnité Inserm U342, Pathologie Métabolique et Hormonale du Developpement, Hôpital St. Vincent de Paul, Paris, France; Unité de Neurovirologie et Régénération du Système Nerveux, Département de Virologie, Institut Pasteur, Paris, France; Laboratory of Viral and Molecular Pathogenesis, National Institutes of Health, Bethesda, Maryland, 20892; Laboratory of Neurobiology, NINDS, National Institutes of Health, Bethesda, Maryland, 20892; Département de Biologie Clinique, Institut Gustave Roussy, Villejuif, France; Amgen, Thousand Oaks, California, 91320; Unité Inserm U29, Hôpital Port-Royal, Paris, FranceUnité Inserm U342, Pathologie Métabolique et Hormonale du Developpement, Hôpital St. Vincent de Paul, Paris, France; Unité de Neurovirologie et Régénération du Système Nerveux, Département de Virologie, Institut Pasteur, Paris, France; Laboratory of Viral and Molecular Pathogenesis, National Institutes of Health, Bethesda, Maryland, 20892; Laboratory of Neurobiology, NINDS, National Institutes of Health, Bethesda, Maryland, 20892; Département de Biologie Clinique, Institut Gustave Roussy, Villejuif, France; Amgen, Thousand Oaks, California, 91320; Unité Inserm U29, Hôpital Port-Royal, Paris, FranceUnité Inserm U342, Pathologie Métabolique et Hormonale du Developpement, Hôpital St. Vincent de Paul, Paris, France; Unité de Neurovirologie et Régénération du Système Nerveux, Département de Virologie, Institut Pasteur, Paris, France; Laboratory of Viral and Molecular Pathogenesis, National Institutes of Health, Bethesda, Maryland, 20892; Laboratory of Neurobiology, NINDS, National Institutes of Health, Bethesda, Maryland, 20892; Département de Biologie Clinique, Institut Gustave Roussy, Villejuif, France; Amgen, Thousand Oaks, California, 91320; Unité Inserm U29, Hôpital Port-Royal, Paris, FranceUnité Inserm U342, Pathologie Métabolique et Hormonale du Developpement, Hôpital St. Vincent de Paul, Paris, France; Unité de Neurovirologie et Régénération du Système Nerveux, Département de Virologie, Institut Pasteur, Paris, France; Laboratory of Viral and Molecular Pathogenesis, National Institutes of Health, Bethesda, Maryland, 20892; Laboratory of Neurobiology, NINDS, National Institutes of Health, Bethesda, Maryland, 20892; Département de Biologie Clinique, Institut Gustave Roussy, Villejuif, France; Amgen, Thousand Oaks, California, 91320; Unité Inserm U29, Hôpital Port-Royal, Paris, FranceUnité Inserm U342, Pathologie Métabolique et Hormonale du Developpement, Hôpital St. Vincent de Paul, Paris, France; Unité de Neurovirologie et Régénération du Système Nerveux, Département de Virologie, Institut Pasteur, Paris, France; Laboratory of Viral and Molecular Pathogenesis, National Institutes of Health, Bethesda, Maryland, 20892; Laboratory of Neurobiology, NINDS, National Institutes of Health, Bethesda, Maryland, 20892; Département de Biologie Clinique, Institut Gustave Roussy, Villejuif, France; Amgen, Thousand Oaks, California, 91320; Unité Inserm U29, Hôpital Port-Royal, Paris, FranceUnité Inserm U342, Pathologie Métabolique et Hormonale du Developpement, Hôpital St. Vincent de Paul, Paris, France; Unité de Neurovirologie et Régénération du Système Nerveux, Département de Virologie, Institut Pasteur, Paris, France; Laboratory of Viral and Molecular Pathogenesis, National Institutes of Health, Bethesda, Maryland, 20892; Laboratory of Neurobiology, NINDS, National Institutes of Health, Bethesda, Maryland, 20892; Département de Biologie Clinique, Institut Gustave Roussy, Villejuif, France; Amgen, Thousand Oaks, California, 91320; Unité Inserm U29, Hôpital Port-Royal, Paris, FranceUnité Inserm U342, Pathologie Métabolique et Hormonale du Developpement, Hôpital St. Vincent de Paul, Paris, France; Unité de Neurovirologie et Régénération du Système Nerveux, Département de Virologie, Institut Pasteur, Paris, France; Laboratory of Viral and Molecular Pathogenesis, National Institutes of Health, Bethesda, Maryland, 20892; Laboratory of Neurobiology, NINDS, National Institutes of Health, Bethesda, Maryland, 20892; Département de Biologie Clinique, Institut Gustave Roussy, Villejuif, France; Amgen, Thousand Oaks, California, 91320; Unité Inserm U29, Hôpital Port-Royal, Paris, FranceThe 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 β-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 cellsin vivoand in regenerating oligodendrocytesin 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.http://www.sciencedirect.com/science/article/pii/S0969996197901273 |
| spellingShingle | Francoise Fouquet Jia Min Zhou Evelyn Ralston Kerren Murray Frédéric Troalen Ella Magal Olivier Robain M. Dubois-Dalcq Patrick Aubourg Expression of the Adrenoleukodystrophy Protein in the Human and Mouse Central Nervous System Neurobiology of Disease |
| title | Expression of the Adrenoleukodystrophy Protein in the Human and Mouse Central Nervous System |
| title_full | Expression of the Adrenoleukodystrophy Protein in the Human and Mouse Central Nervous System |
| title_fullStr | Expression of the Adrenoleukodystrophy Protein in the Human and Mouse Central Nervous System |
| title_full_unstemmed | Expression of the Adrenoleukodystrophy Protein in the Human and Mouse Central Nervous System |
| title_short | Expression of the Adrenoleukodystrophy Protein in the Human and Mouse Central Nervous System |
| title_sort | expression of the adrenoleukodystrophy protein in the human and mouse central nervous system |
| url | http://www.sciencedirect.com/science/article/pii/S0969996197901273 |
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