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Plasma polyenoic very-long-chain fatty acids in peroxisomal disease

Biochemical discrimination of Zellweger's syndrome from other phenotypes

Department of Chemical Pathology, Adelaide Children's Hospital, North Adelaide, South Australia.

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.

Address correspondence and reprint requests to Dr. Poulos, Department of Chemical Pathology, Adelaide Children's Hospital, 72 King William Road, North Adelaide, South Australia 5006, Australia.

Supported in part by a grant to D.W.J. from the National Health and Medical Research Council of Australia.

Received February 29, 1988. Accepted for publication in final form July 12, 1988.