Molecular Species of Phosphatidylcholine Containing Very Long Chain Fatty Acids in Human Brain: Enrichment in X‐Linked Adrenoleukodystrophy Brain and Diseases of Peroxisome Biogenesis Brain
作者:
P. Sharp,
D. Johnson,
A. Poulos,
期刊:
Journal of Neurochemistry
(WILEY Available online 1991)
卷期:
Volume 56,
issue 1
页码: 30-37
ISSN:0022-3042
年代: 1991
DOI:10.1111/j.1471-4159.1991.tb02558.x
出版商: Blackwell Publishing Ltd
关键词: Brain;Zellweger's syndrome;Peroxisomes;Phosphatidylcholine;Polyenoic very long chain fatty acids
数据来源: WILEY
摘要:
Abstract:Molecular species of phosphatidylcholine containing unsaturated (i.e., monoenoic and polyenoic) 32‐ to 40‐carbon (very long chain) fatty acids (VLCFA‐PC) are present in normal human brain, the fatty acid composition changing significantly with development. There is a marked increase in the concentration and a change in the polyenoic VLCFA composition of these molecular species in brains of patients with inherited defects in peroxisomal biogenesis [Zellweger's syndrome, neonatal adrenoleukodystrophy (ALD), and infantile Refsum's disease]. In contrast, there is a marked increase in monoenoic VLCFA‐PC in X‐linked ALD whereas molecular species containing polyenoic VLCFA are minor c
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