Purification and characterization of branched chain alpha-keto acid dehydrogenase complex of bovine kidney
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Solution structure and dynamics of the lipoic acid-bearing domain of human mitochondrial branched-chain alpha-keto acid dehydrogenase complexMolecular and biochemical basis of intermediate maple syrup urine disease. Occurrence of homozygous G245R and F364C mutations at the E1 alpha locus of Hispanic-Mexican patientsOxidative decarboxylation of 4-methylthio-2-oxobutyrate by branched-chain 2-oxo acid dehydrogenase complexActivities 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: identification and carrier-frequency determination of a novel founder mutation in the Ashkenazi Jewish population.Maple 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 diseaseEvidence for both a regulatory mutation and a structural mutation in a family with maple syrup urine 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 disPurification and properties of branched-chain alpha-keto acid dehydrogenase phosphatase from bovine kidneyCloning and cDNA sequence of the dihydrolipoamide dehydrogenase component human alpha-ketoacid dehydrogenase complexesLipoic acid metabolism of Plasmodium--a suitable drug targetStructure of rat BCKD kinase: Nucleotide-induced domain communication in a mitochondrial protein kinaseStructural and Thermodynamic Basis for Weak Interactions between Dihydrolipoamide Dehydrogenase and Subunit-binding Domain of the Branched-chain -Ketoacid Dehydrogenase ComplexDistribution of key enzymes of branched-chain amino acid metabolism in glial and neuronal cells in cultureThe C-terminal hinge region of lipoic acid-bearing domain of E2b is essential for domain interaction with branched-chain alpha-keto acid dehydrogenase kinasePremature termination of transcription and alternative splicing in the human transacylase (E2) gene of the branched-chain alpha-ketoacid dehydrogenase complexThe 2-oxo acid dehydrogenase complexes: recent advancesAltered 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 syAcute regulation of the branched-chain 2-oxo acid dehydrogenase complex by adrenaline and glucagon in the perfused rat heartRole of activation of myocardial branched-chain 2-oxo acid dehydrogenase complex in the regulation of leucine decarboxylation during cardiac work in vitroBovine serum albumin decreases 4-methyl-2-oxovalerate utilization by isolated rat hepatocytesDevelopmental changes in rat liver branched-chain 2-oxo acid dehydrogenaseProtein markers for insulin-producing beta cells with higher glucose sensitivityThe bkdR gene of Pseudomonas putida is required for expression of the bkd operon and encodes a protein related to Lrp of Escherichia coliRoles of active site and novel K+ ion-binding site residues in human mitochondrial branched-chain alpha-ketoacid decarboxylase/dehydrogenase.Role of bkdR, a transcriptional activator of the sigL-dependent isoleucine and valine degradation pathway in Bacillus subtilisCatabolism of branched-chain alpha-keto acids in Enterococcus faecalis: the bkd gene cluster, enzymes, and metabolic route.Alveolate mitochondrial metabolic evolution: dinoflagellates force reassessment of the role of parasitism as a driver of change in apicomplexans.Maple syrup urine disease in Cypriot families: identification of three novel mutations and biochemical characterization of the p.Thr211Met mutation in the E1alpha subunit.Circulating cortisol-associated signature of glucocorticoid-related gene expression in subcutaneous fat of obese subjects.Mitochondrial metabolism of sexual and asexual blood stages of the malaria parasite Plasmodium falciparum.Interaction of peroxiredoxin V with dihydrolipoamide branched chain transacylase E2 (DBT) in mouse kidney under hypoxia.Detection of heterozygotes in maple-syrup-urine disease: measurements of branched-chain alpha-ketoacid dehydrogenase and its components in cell cultures.A synchronized substrate-gating mechanism revealed by cubic-core structure of the bovine branched-chain alpha-ketoacid dehydrogenase complexEmerging perspectives on essential amino acid metabolism in obesity and the insulin-resistant state.Binding of L-branched-chain amino acids causes a conformational change in BkdR.Molecular cloning of genes encoding branched-chain keto acid dehydrogenase of Pseudomonas putida.Thiamin-responsive maple-syrup-urine disease: decreased affinity of the mutant branched-chain alpha-keto acid dehydrogenase for alpha-ketoisovalerate and thiamin pyrophosphate.Isolation of a specific lipoamide dehydrogenase for a branched-chain keto acid dehydrogenase from Pseudomonas putidaPurification of a branched-chain keto acid dehydrogenase from Pseudomonas putida.
P2860
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P2860
Purification and characterization of branched chain alpha-keto acid dehydrogenase complex of bovine kidney
description
1978 nî lūn-bûn
@nan
1978 թուականի Հոկտեմբերին հրատարակուած գիտական յօդուած
@hyw
1978 թվականի հոտեմբերին հրատարակված գիտական հոդված
@hy
1978年の論文
@ja
1978年学术文章
@wuu
1978年学术文章
@zh-cn
1978年学术文章
@zh-hans
1978年学术文章
@zh-my
1978年学术文章
@zh-sg
1978年學術文章
@yue
name
Purification and characterizat ...... enase complex of bovine kidney
@ast
Purification and characterizat ...... enase complex of bovine kidney
@en
Purification and characterizat ...... enase complex of bovine kidney
@nl
type
label
Purification and characterizat ...... enase complex of bovine kidney
@ast
Purification and characterizat ...... enase complex of bovine kidney
@en
Purification and characterizat ...... enase complex of bovine kidney
@nl
prefLabel
Purification and characterizat ...... enase complex of bovine kidney
@ast
Purification and characterizat ...... enase complex of bovine kidney
@en
Purification and characterizat ...... enase complex of bovine kidney
@nl
P2093
P2860
P356
P1476
Purification and characterizat ...... enase complex of bovine kidney
@en
P2093
P2860
P304
P356
10.1073/PNAS.75.10.4881
P407
P577
1978-10-01T00:00:00Z