Myelin Membrane from Adrenoleukodystrophy Brain White Matter—Biochemical Properties

Frank R. Brown; Winston W. Chen; Daniel A. Kirschner; Karen L. Frayer; James M. Powers; Ann B. Moser; Hugo W. Moser

doi:10.1111/j.1471-4159.1983.tb04748.x

Myelin Membrane from Adrenoleukodystrophy Brain White Matter—Biochemical Properties

Frank R. Brown, Winston W. Chen, Daniel A. Kirschner, Karen L. Frayer, James M. Powers, Ann B. Moser, Hugo W. Moser

Research output: Contribution to journal › Article › peer-review

35 Scopus citations

Abstract

Abstract: Adrenoleukodystrophy (ALD) is an X‐linked progressive neurological disorder characterized by the accumulation of saturated very‐long‐chain fatty acids (C₂₄ to C₃₀) in lipids, especially cholesterol esters of tne brain white matter and adrenal cortex. In the present study we have investigated the localization of accumulated cholesterol esters in brain white matter. During isolation of purified myelin membrane from regions of active demyelination, significant enrichment in cholesterol ester was found in two fractions, mainly in a low‐density floating fraction and to a lesser degree in the purified myelin preparation. The fatty acid composition of cholesterol esters from both the ALD floating and myelin fractions was enriched approximately 10‐fold in saturated very‐long‐chain fatty acids (≥C24) compared with control preparations.

Original language	English (US)
Pages (from-to)	341-348
Number of pages	8
Journal	Journal of Neurochemistry
Volume	41
Issue number	2
DOIs	https://doi.org/10.1111/j.1471-4159.1983.tb04748.x
State	Published - Feb 1983
Externally published	Yes

Keywords

Adrenoleukodystrophy
Cholesterol ester
Electron microscopy
Fatty acids
Myelin

ASJC Scopus subject areas

Biochemistry
Cellular and Molecular Neuroscience

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10.1111/j.1471-4159.1983.tb04748.x

Cite this

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title = "Myelin Membrane from Adrenoleukodystrophy Brain White Matter—Biochemical Properties",

abstract = "Abstract: Adrenoleukodystrophy (ALD) is an X‐linked progressive neurological disorder characterized by the accumulation of saturated very‐long‐chain fatty acids (C24 to C30) in lipids, especially cholesterol esters of tne brain white matter and adrenal cortex. In the present study we have investigated the localization of accumulated cholesterol esters in brain white matter. During isolation of purified myelin membrane from regions of active demyelination, significant enrichment in cholesterol ester was found in two fractions, mainly in a low‐density floating fraction and to a lesser degree in the purified myelin preparation. The fatty acid composition of cholesterol esters from both the ALD floating and myelin fractions was enriched approximately 10‐fold in saturated very‐long‐chain fatty acids (≥C24) compared with control preparations.",

keywords = "Adrenoleukodystrophy, Cholesterol ester, Electron microscopy, Fatty acids, Myelin",

author = "Brown, {Frank R.} and Chen, {Winston W.} and Kirschner, {Daniel A.} and Frayer, {Karen L.} and Powers, {James M.} and Moser, {Ann B.} and Moser, {Hugo W.}",

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AU - Brown, Frank R.

AU - Chen, Winston W.

AU - Kirschner, Daniel A.

AU - Frayer, Karen L.

AU - Powers, James M.

AU - Moser, Ann B.

AU - Moser, Hugo W.

PY - 1983/2

Y1 - 1983/2

N2 - Abstract: Adrenoleukodystrophy (ALD) is an X‐linked progressive neurological disorder characterized by the accumulation of saturated very‐long‐chain fatty acids (C24 to C30) in lipids, especially cholesterol esters of tne brain white matter and adrenal cortex. In the present study we have investigated the localization of accumulated cholesterol esters in brain white matter. During isolation of purified myelin membrane from regions of active demyelination, significant enrichment in cholesterol ester was found in two fractions, mainly in a low‐density floating fraction and to a lesser degree in the purified myelin preparation. The fatty acid composition of cholesterol esters from both the ALD floating and myelin fractions was enriched approximately 10‐fold in saturated very‐long‐chain fatty acids (≥C24) compared with control preparations.

AB - Abstract: Adrenoleukodystrophy (ALD) is an X‐linked progressive neurological disorder characterized by the accumulation of saturated very‐long‐chain fatty acids (C24 to C30) in lipids, especially cholesterol esters of tne brain white matter and adrenal cortex. In the present study we have investigated the localization of accumulated cholesterol esters in brain white matter. During isolation of purified myelin membrane from regions of active demyelination, significant enrichment in cholesterol ester was found in two fractions, mainly in a low‐density floating fraction and to a lesser degree in the purified myelin preparation. The fatty acid composition of cholesterol esters from both the ALD floating and myelin fractions was enriched approximately 10‐fold in saturated very‐long‐chain fatty acids (≥C24) compared with control preparations.

KW - Adrenoleukodystrophy

KW - Cholesterol ester

KW - Electron microscopy

KW - Fatty acids

KW - Myelin

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Myelin Membrane from Adrenoleukodystrophy Brain White Matter—Biochemical Properties

Abstract

Keywords

ASJC Scopus subject areas

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