Plasma polyenoic very‐long‐chain fatty acids in peroxisomal disease
摘要
The plasma of patients with inherited defects in peroxisomal biogenesis (ie, Zellweger's syndrome, infantile Refsum's disease, and neonatal adrenoleukodystrophy) shows evidence of a disturbance in the metabolism of saturated and monoenoic fatty acids with carbon chain lengths greater than 22 (VLCFA). Zellweger's syndrome plasma alone contains, in addition, increased amounts of a number of n-6 polyenoic VLCFA including 24:5, 26:5, 28:5, 30:5, and 30:6 fatty acids. These fatty acids facilitate the biochemical discrimination of Zellweger's syndrome from other related phenotypes.
引用本文（GB/T 7714）
A. Poulos, P. Sharp, Detra D. Johnson. Plasma polyenoic very‐long‐chain fatty acids in peroxisomal disease[J]. Neurology, 1989.
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