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Solution structure and dynamics of the lipoic acid-bearing domain of human mitochondrial branched-chain alpha-keto acid dehydrogenase complexMaple syrup urine disease. Complete defect of the E1 beta subunit of the branched chain alpha-ketoacid dehydrogenase complex due to a deletion of an 11-bp repeat sequence which encodes a mitochondrial targeting leader peptide in a family with the diMolecular and biochemical basis of intermediate maple syrup urine disease. Occurrence of homozygous G245R and F364C mutations at the E1 alpha locus of Hispanic-Mexican patientsCloning and nucleotide sequence of the cDNA encoding human 2-oxoglutarate dehydrogenase (lipoamide)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 diseaseMaple syrup urine disease in Mennonites. Evidence that the Y393N mutation in E1 alpha impedes assembly of the E1 component of branched-chain alpha-keto acid dehydrogenase complexCatalytic domain of PDC-E2 contains epitopes recognized by antimitochondrial antibodies in primary biliary cirrhosisOccurrence of a 2-bp (AT) deletion allele and a nonsense (G-to-T) mutant allele at the E2 (DBT) locus of six patients with maple syrup urine disease: multiple-exon skipping as a secondary effect of the mutationsMolecular basis of maple syrup urine disease: novel mutations at the E1 alpha locus that impair E1(alpha 2 beta 2) assembly or decrease steady-state E1 alpha mRNA levels of branched-chain alpha-keto acid dehydrogenase complexMaple syrup urine disease: the E1beta gene of human branched-chain alpha-ketoacid dehydrogenase complex has 11 rather than 10 exons, and the 3' UTR in one of the two E1beta mRNAs arises from intronic sequencesOccurrence of a Tyr393----Asn (Y393N) mutation in the E1 alpha gene of the branched-chain alpha-keto acid dehydrogenase complex in maple syrup urine disease patients from a Mennonite populationStructural and Thermodynamic Basis for Weak Interactions between Dihydrolipoamide Dehydrogenase and Subunit-binding Domain of the Branched-chain -Ketoacid Dehydrogenase ComplexStructural and Biochemical Characterization of Human Mitochondrial Branched-chain -Ketoacid Dehydrogenase PhosphatasePyruvate decarboxylase catalyzes decarboxylation of branched-chain 2-oxo acids but is not essential for fusel alcohol production by Saccharomyces cerevisiae.Yeast pyruvate dehydrogenase complex is regulated by a concerted activity of two kinases and two phosphatases.In vivo assembly of yeast mitochondrial alpha-ketoglutarate dehydrogenase complexTetrameric assembly and conservation in the ATP-binding domain of rat branched-chain alpha-ketoacid dehydrogenase kinaseIsolation, characterization and structural organization of the gene and pseudogene for the dihydrolipoamide succinyltransferase component of the human 2-oxoglutarate dehydrogenase complexThe role of amino acids T148 and R281 in human dihydrolipoamide dehydrogenaseThe role of N286 and D320 in the reaction mechanism of human dihydrolipoamide dehydrogenase (E3) center domainAltered phosphorylation state of branched-chain 2-oxo acid dehydrogenase in a branched-chain acyltransferase deficient human fibroblast cell lineGene preference in maple syrup urine disease.Do mammalian cells synthesize lipoic acid? Identification of a mouse cDNA encoding a lipoic acid synthase located in mitochondriaRole of pyruvate dehydrogenase kinase isoenzyme 4 (PDHK4) in glucose homoeostasis during starvationProteomic analysis of carbon concentrating chemolithotrophic bacteria Serratia sp. for sequestration of carbon dioxideConformational stability and thermodynamic characterization of the lipoic acid bearing domain of human mitochondrial branched chain alpha-ketoacid dehydrogenase.Folding kinetics of the lipoic acid-bearing domain of human mitochondrial branched chain alpha-ketoacid dehydrogenase complex.cDNA cloning of the chicken branched-chain alpha-keto acid dehydrogenase complex. Chicken-specific residues of the acyltransferase affect the overall activity and the interaction with the dehydrogenase.Isolation and characterization of cDNA clones for the e1beta and E2 subunits of the branched-chain alpha-ketoacid dehydrogenase complex in Arabidopsis.Two-way learning with one-way supervision for gene expression data.Roles of active site and novel K+ ion-binding site residues in human mitochondrial branched-chain alpha-ketoacid decarboxylase/dehydrogenase.Potential biomarkers for ischemic heart damage identified in mitochondrial proteins by comparative proteomics.Production and characterization of murine models of classic and intermediate maple syrup urine diseaseLens metabolomic profiling as a tool to understand cataractogenesis in Atlantic salmon and rainbow trout reared at optimum and high temperatureReactivity of primary biliary cirrhosis sera with Escherichia coli dihydrolipoamide acetyltransferase (E2p): characterization of the main immunogenic region.Lipoic acid metabolism in Arabidopsis thaliana: cloning and characterization of a cDNA encoding lipoyltransferase.Differential expression profile of membrane proteins in zebrafish (Danio rerio) brain exposed to methyl parathion.Molecular cloning of the gene for the E1 alpha subunit of the pyruvate dehydrogenase complex from Saccharomyces cerevisiae.Purification and properties of the lipoate protein ligase of Escherichia coliAutoantigens in primary biliary cirrhosis
P2860
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P2860
description
1989 nî lūn-bûn
@nan
1989 թուականի Փետրուարին հրատարակուած գիտական յօդուած
@hyw
1989 թվականի փետրվարին հրատարակված գիտական հոդված
@hy
1989年の論文
@ja
1989年論文
@yue
1989年論文
@zh-hant
1989年論文
@zh-hk
1989年論文
@zh-mo
1989年論文
@zh-tw
1989年论文
@wuu
name
The 2-oxo acid dehydrogenase complexes: recent advances
@ast
The 2-oxo acid dehydrogenase complexes: recent advances
@en
The 2-oxo acid dehydrogenase complexes: recent advances
@nl
type
label
The 2-oxo acid dehydrogenase complexes: recent advances
@ast
The 2-oxo acid dehydrogenase complexes: recent advances
@en
The 2-oxo acid dehydrogenase complexes: recent advances
@nl
prefLabel
The 2-oxo acid dehydrogenase complexes: recent advances
@ast
The 2-oxo acid dehydrogenase complexes: recent advances
@en
The 2-oxo acid dehydrogenase complexes: recent advances
@nl
P2860
P3181
P356
P1433
P1476
The 2-oxo acid dehydrogenase complexes: recent advances
@en
P2093
S J Yeaman
P2860
P304
P3181
P356
10.1042/BJ2570625
P407
P577
1989-02-01T00:00:00Z