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Myelin Membrane from Adrenoleukodystrophy Brain White Matter—Biochemical Properties
Author(s) -
Brown Frank R.,
Chen Winston W.,
Kirschner Daniel A.,
Frayer Karen L.,
Powers James M.,
Moser Ann B.,
Moser Hugo W.
Publication year - 1983
Publication title -
journal of neurochemistry
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.75
H-Index - 229
eISSN - 1471-4159
pISSN - 0022-3042
DOI - 10.1111/j.1471-4159.1983.tb04748.x
Subject(s) - adrenoleukodystrophy , myelin , white matter , cholesterol , chemistry , biochemistry , membrane , fatty acid , chromatography , biology , endocrinology , central nervous system , medicine , magnetic resonance imaging , peroxisome , radiology , gene
Adrenoleukodystrophy (ALD) is an X‐linked progressive neurological disorder characterized by the accumulation of saturated very‐long‐chain fatty acids (C 24 to C 30 ) 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.