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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Borud, O., Pettersen, J.E. Normal 2-aminobutyrate oxidation and increased valine oxidation in fibroblasts deficient in pyruvate dehydrogenase. J Inherit Metab Dis 5, 55–57 (1982). https://doi.org/10.1007/BF01799755
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DOI: https://doi.org/10.1007/BF01799755