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Peroxisomal l-pipecolic acid oxidation is deficient in liver from zellweger syndrome patients

Pediatric Research (1989) 25(5) 548-552
DOI: 10.1203/00006450-198905000-00024
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Abstract

L-Pipecolic acid, a cyclic imino acid produced during the degradation of lysine, accumulates in body fluids of infants with the generalized peroxisomal disorders, including Zellweger syndrome, neonatal adrenoleukodystrophy, and infantile Refsum disease. Peroxisome-enriched fractions from normal human liver oxidized L-[3H]pipecolic acid to

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Mihalik, S. J., Moser, H. W., Watkins, P. A., Danks, D. M., Poulos, A., & Rhead, W. J. (1989). Peroxisomal l-pipecolic acid oxidation is deficient in liver from zellweger syndrome patients. Pediatric Research, 25(5), 548–552. https://doi.org/10.1203/00006450-198905000-00024

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