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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
Author(s) -
Sharp P.,
Johnson D.,
Poulos A.
Publication year - 1991
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.1471-4159.1991.tb02558.x
Subject(s) - adrenoleukodystrophy , peroxisome , phosphatidylcholine , biogenesis , zellweger syndrome , biochemistry , biology , peroxisomal disorder , fatty acid , phospholipid , gene , membrane
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 components.