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Localization of pipecolic acid metabolism in rat liver peroxisomes: Probable explanation for hyperpipecolataemia in Zellweger syndrome
Author(s) -
Trijbels J. M. F.,
Monnens L. A. H.,
Melis G.,
Broekvan Essen M.,
Bruckwilder M.
Publication year - 1987
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1007/bf01800037
Subject(s) - pipecolic acid , peroxisome , zellweger syndrome , biochemistry , metabolism , percoll , biology , microbody , enzyme , differential centrifugation , oxidase test , peroxisomal disorder , centrifugation , chemistry , amino acid , receptor
The metabolism of [ 14 C]pipecolic acid was studied in peroxisomal fractions of rat liver obtained by density gradient centrifugation in Percoll. The production rate of [ 14 CO 2 ] was used to measure the metabolic activity of the fractions towards [ 14 C]carboxypipecolic acid as a substrate. It was shown that this activity was located in the peroxisomal fractions by comparison with the peroxisomal marker enzyme urate oxidase (EC 1.7.3.3). The process was markedly elevated by the addition of FAD. The apparent K m for DL ‐pipecolic acid was found to be 1.2 mmol L −1 . Addition of ATP (1 mmol L −1 ) did not influence the decarboxylation rate of pipecolic acid. These results might explain the defective metabolism of pipecolic acid in patients with Zellweger syndrome who are lacking peroxisomes.