Purification and characterization of branched chain alpha-ketoacid dehydrogenase from bovine liver mitochondria

Purification and characterization of branched chain alpha-ketoacid dehydrogenase from bovine liver mitochondria

Item
http://www.wikidata.org/entity/Q28242070
Activities of branched-chain 2-oxo acid dehydrogenase and its components in skin fibroblasts from normal and classical-maple-syrup-urine-disease subjectsMaple syrup urine disease. Complete primary structure of the E1 beta subunit of human branched chain alpha-ketoacid dehydrogenase complex deduced from the nucleotide sequence and a gene analysis of patients with this diseaseMaple syrup urine disease caused by a partial deletion in the inner E2 core domain of the branched chain alpha-keto acid dehydrogenase complex due to aberrant splicing. A single base deletion at a 5'-splice donor site of an intron of the E2 gene disAltered kinetic properties of the branched-chain alpha-keto acid dehydrogenase complex due to mutation of the beta-subunit of the branched-chain alpha-keto acid decarboxylase (E1) component in lymphoblastoid cells derived from patients with maple syPhenylbutyrate therapy for maple syrup urine disease.Detection of heterozygotes in maple-syrup-urine disease: measurements of branched-chain alpha-ketoacid dehydrogenase and its components in cell cultures.Thiamin-responsive maple-syrup-urine disease: decreased affinity of the mutant branched-chain alpha-keto acid dehydrogenase for alpha-ketoisovalerate and thiamin pyrophosphate.Purification of a branched-chain keto acid dehydrogenase from Pseudomonas putida.Direct physical evidence for stabilization of branched-chain alpha-ketoacid dehydrogenase by thiamin pyrophosphate.Reversible ATP-induced inactivation of branched-chain 2-oxo acid dehydrogenase.Dual role of a single multienzyme complex in the oxidative decarboxylation of pyruvate and branched-chain 2-oxo acids in Bacillus subtilis.A study of the metabolism of [U-14C]3-methyl-2-oxopentanoate by rat liver mitochondria using h.p.l.c. with continuous on-line monitoring of radioactive intact acyl-coenzyme A intermediates.The regulation of branched-chain 2-oxo acid dehydrogenase of liver, kidney and heart by phosphorylation.Purification of rat kidney branched-chain oxo acid dehydrogenase complex with endogenous kinase activity.Antibodies to bovine liver branched-chain 2-oxo acid dehydrogenase cross-react with this enzyme complex from other tissues and species.An improved assay for pyruvate dehydrogenase: reply.
P2860

Purification and characterization of branched chain alpha-ketoacid dehydrogenase from bovine liver mitochondria

Item
http://www.wikidata.org/entity/Q28242070
description
1979 nî lūn-bûn
@nan
1979 թուականի Յունիսին հրատարակուած գիտական յօդուած
@hyw
1979 թվականի հունիսին հրատարակված գիտական հոդված
@hy
1979年の論文
@ja
1979年論文
@yue
1979年論文
@zh-hant
1979年論文
@zh-hk
1979年論文
@zh-mo
1979年論文
@zh-tw
1979年论文
@wuu
name
Purification and characterizat ...... from bovine liver mitochondria
@ast
Purification and characterizat ...... from bovine liver mitochondria
@en
Purification and characterizat ...... from bovine liver mitochondria
@nl
type
label
Purification and characterizat ...... from bovine liver mitochondria
@ast
Purification and characterizat ...... from bovine liver mitochondria
@en
Purification and characterizat ...... from bovine liver mitochondria
@nl
prefLabel
Purification and characterizat ...... from bovine liver mitochondria
@ast
Purification and characterizat ...... from bovine liver mitochondria
@en
Purification and characterizat ...... from bovine liver mitochondria
@nl
P1433
P1476
P2093
P304
P31
P407
P433
P478
P577
P698
P921
P1433
P1476
Purification and characterizat ...... from bovine liver mitochondria
@en
P2093
P304
P31
P407
P433
P478
P577
P698
P921