title: Normal 2-aminobutyrate oxidation and increased valine oxidation in fibroblasts deficient in pyruvate dehydrogenase.
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.
mesh_terms: D000613:Aminobutyrates / Q000378:metabolism*; D002245:Carbon Dioxide / Q000378:metabolism; D002478:Cells, Cultured; D005347:Fibroblasts / Q000378:metabolism; D006801:Humans; D010084:Oxidation-Reduction; D015325:Pyruvate Dehydrogenase Complex Deficiency Disease; D013997:Time Factors; D014633:Valine / Q000378:metabolism*
chemical_list: D000613:Aminobutyrates; D002245:Carbon Dioxide; C012223:alpha-aminobutyric acid; D014633:Valine