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Literature DB >> 6475955

Direct physical evidence for stabilization of branched-chain alpha-ketoacid dehydrogenase by thiamin pyrophosphate.

S C Heffelfinger, E T Sewell, L J Elsas, D J Danner.

Abstract

Branched-chain alpha-ketoacid dehydrogenase is a multienzyme complex composed of four subunits. The 46,500-dalton protein is a subunit of the decarboxylase component, which is selectively digested by chymotrypsin. Two peptides of apparent mol. wts. of 36,000 and 15,000 result with loss of enzyme activity. When the complex is saturated with thiamin pyrophosphate and ketoacid substrate, digestion by chymotrypsin does not occur. These data provide direct physical evidence for the stabilization of the complex by the presence of the vitamin B1-derived cofactor.

Entities: Chemical

Mesh：

Substances：

Year: 1984 PMID： 6475955 PMCID： PMC1684491

Source DB: PubMed Journal: Am J Hum Genet ISSN： 0002-9297 Impact factor: 11.025

17 in total

1. Thiamine increases the specific activity of human liver branched chain alpha-ketoacid dehydrogenase.

Authors: D J Danner; E D Davidson; L J Elsas
Journal: Nature Date: 1975-04-10 Impact factor: 49.962

2. THE COFACTOR-MEDIATED REGULATION OF APOENZYME LEVELS IN ANIMAL TISSUES. I. THE PYRIDOXINE-INDUCED RISE OF RAT LIVER TYROSINE TRANSAMINASE LEVEL IN VIVO.

Authors: O GREENGARD; M GORDON
Journal: J Biol Chem Date: 1963-11 Impact factor: 5.157

3. Thiamine-responsive maple-syrup-urine disease.

Authors: C R Scriver; S Mackenzie; C L Clow; E Delvin
Journal: Lancet Date: 1971-02-13 Impact factor: 79.321

4. Tyrosine aminotransferase. Purificaton and characterization.

Authors: S I Hayashi; D K Granner; G M Tomkins
Journal: J Biol Chem Date: 1967-09-25 Impact factor: 5.157

5. Regulation of leucine and alpha-ketoisocaproate metabolism in skeletal muscle.

Authors: S M Hutson; T C Cree; A E Harper
Journal: J Biol Chem Date: 1978-11-25 Impact factor: 5.157

6. In vivo and in vitro response of human branched chain alpha-ketoacid dehydrogenase to thiamine and thiamine pyrophosphate.

Authors: D J Danner; F B Wheeler; S K Lemmon; L J Elsas
Journal: Pediatr Res Date: 1978-03 Impact factor: 3.756

7. Regulation of branched-chain amino acid oxidation in isolated muscles, nerves and aortas of rats.

Authors: M G Buse; S Jursinic; S S Reid
Journal: Biochem J Date: 1975-06 Impact factor: 3.857

8. Effects of thiamine in a patient with a variant form of branched-chian ketoaciduria.

Authors: M Duran; A G Tielens; S K Wadman; J C Stigter; W J Kleijer
Journal: Acta Paediatr Scand Date: 1978-05

9. On the mechanism of pyridoxine responsive homocystinuria. II. Properties of normal and mutant cystathionine beta-synthase from cultured fibroblasts.

Authors: Y J Kim; L E Rosenberg
Journal: Proc Natl Acad Sci U S A Date: 1974-12 Impact factor: 11.205

10. Cystathionine beta-synthase deficiency: a qualitative abnormality of the deficient enzyme modified by vitamin B6 therapy.

Authors: R C Longhi; L D Fleisher; H H Tallan; G E Gaull
Journal: Pediatr Res Date: 1977-02 Impact factor: 3.756

4 in total

1. Sequence of the E1 alpha subunit of branched-chain alpha-ketoacid dehydrogenase in two patients with thiamine-responsive maple syrup urine disease.

Authors: B Zhang; R S Wappner; I K Brandt; R A Harris; D W Crabb
Journal: Am J Hum Genet Date: 1990-04 Impact factor: 11.025