Normal 2‐aminobutyrate oxidation and increased valine oxidation in fibroblasts deficient in pyruvate dehydrogenase

1 March 1982

journal article
research article
Published by Wiley in Journal of Inherited Metabolic Disease

Vol. 5 (1) , 55-57
https://doi.org/10.1007/bf01799755

Abstract

Human skin fibroblasts deficient in pyruvate dehydrogenase and five normal control strains were incubated with one of the following labelled substrates:dl-[1-¹⁴C]-2-amino-n-butyric acid,dl-[3-¹⁴C]-2-amino-n-butyric acid,dl-[1-¹⁴C]leucine,l-[1-¹⁴C]valine,l-[1-¹⁴C]alanine, and [1-¹⁴C]pyruvate. The rate of¹⁴CO₂-production in the deficient cells was normal from 2-aminobutyrate and leucine, increased from valine, and decreased from alanine and pyruvate. These results indicated that in human skin fibroblasts the decarboxylation of 2-oxobutyrate is catalysed by an enzyme system different from the pyruvate dehydrogenase complex.

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