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.
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.
Department of Chemical Pathology, Adelaide Medical Center for Women and Children, Australia.
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Johnson
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Poulos
D000293:Adolescent; D000326:Adrenoleukodystrophy / Q000235:genetics / Q000378:metabolism*; D000368:Aged; D000369:Aged, 80 and over; D001921:Brain / Q000196:embryology / Q000378:metabolism*; D001923:Brain Chemistry; D002648:Child; D002675:Child, Preschool; D002851:Chromatography, High Pressure Liquid; D004075:Diglycerides / Q000378:metabolism; D005227:Fatty Acids / Q000032:analysis / Q000737:chemistry / Q000378:metabolism*; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D009682:Magnetic Resonance Spectroscopy; D008297:Male; D008830:Microbodies / Q000378:metabolism; D008875:Middle Aged; D010713:Phosphatidylcholines / Q000032:analysis / Q000737:chemistry / Q000378:metabolism*; D010741:Phospholipases A / Q000378:metabolism; D012035:Refsum Disease / Q000378:metabolism*; D014960:X Chromosome; D015211:Zellweger Syndrome / Q000378:metabolism*
D004075:Diglycerides; D005227:Fatty Acids; D010713:Phosphatidylcholines; D010741:Phospholipases A