Literature DB >> 6820415

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

O Borud, J E Pettersen.   

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, L-[1-14C]leucine, L-[1-14C]valine, L-[1-14C]alanine, and [1-14C]pyruvate. The rate of 14CO2-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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Year:  1982        PMID： 6820415     DOI： 10.1007/bf01799755

Source DB:  PubMed          Journal:  J Inherit Metab Dis        ISSN： 0141-8955            Impact factor:   4.982


  14 in total

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10.  Metabolic studies on normal and pyruvate dehydrogenase deficient cultured human fibroblasts.

Authors:  O Borud; J H Stroomme
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  3 in total

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