Normal 2‐aminobutyrate oxidation and increased valine oxidation in fibroblasts deficient in pyruvate dehydrogenase
| Autor: | J. E. Pettersen, Olav Borud |
|---|---|
| Rok vydání: | 1982 |
| Předmět: |
Pyruvate decarboxylation
Time Factors Pyruvate dehydrogenase kinase Chemistry Aminobutyrates Valine Carbon Dioxide Fibroblasts Pyruvate dehydrogenase phosphatase Pyruvate dehydrogenase complex Pyruvate carboxylase Biochemistry Genetics Humans Dihydrolipoyl transacetylase Branched-chain alpha-keto acid dehydrogenase complex Oxoglutarate dehydrogenase complex Oxidation-Reduction Pyruvate Dehydrogenase Complex Deficiency Disease Cells Cultured Genetics (clinical) |
| Zdroj: | Journal of Inherited Metabolic Disease. 5:55-57 |
| ISSN: | 1573-2665 0141-8955 |
| DOI: | 10.1007/bf01799755 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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