Protein Variants | Comment | Organism |
---|---|---|
additional information | enzyme complex regulation by reversible phosphorylation is impaired in component E1b S292 and S302 mutants, no or residual acylation of the lipoic acid-bearing domain of component E2b, overview | Homo sapiens |
S292D | site-directed mutagenesis, mutation of a alpha-subunit residue of complex component E1b, reduced activity compared to the wild-type E1b component | Homo sapiens |
S292E | site-directed mutagenesis, mutation of a alpha-subunit residue of complex component E1b, highly reduced activity compared to the wild-type E1b component | Homo sapiens |
S292N | site-directed mutagenesis, mutation of a alpha-subunit residue of complex component E1b, reduced activity compared to the wild-type E1b component | Homo sapiens |
S292Q | site-directed mutagenesis, mutation of a alpha-subunit residue of complex component E1b, inactive mutant | Homo sapiens |
S302A | site-directed mutagenesis, mutation of a alpha-subunit residue of complex component E1b, increased activity compared to the wild-type E1b component | Homo sapiens |
KM Value [mM] | KM Value Maximum [mM] | Substrate | Comment | Organism | Structure |
---|---|---|---|---|---|
additional information | - |
additional information | kinetics, wild-type and mutant E1b components | Homo sapiens | |
0.028 | - |
2-oxo-isovalerate | E1b component mutant S302Aalpha | Homo sapiens | |
0.048 | - |
2-oxo-isovalerate | wild-type E1b component | Homo sapiens | |
0.193 | - |
2-oxo-isovalerate | E1b component mutant S292Nalpha | Homo sapiens | |
4.38 | - |
2-oxo-isovalerate | E1b component mutant S292Dalpha | Homo sapiens |
Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
mitochondrion | - |
Homo sapiens | 5739 | - |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
additional information | Homo sapiens | branched-chain alpha-ketoacid dehydrogenase multienzyme complex BCKDC which is regulated by phosphorylation of component E1b, residue Ser292 of the alpha-domain, in response to hormone and dietary stimuli, phosphorylation induces a conformational change of E1b phosphorylation loop which prevents binding of the E2b component, thiamine diphosphate and His291alpha are involved, overview | ? | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
Posttranslational Modification | Comment | Organism |
---|---|---|
phosphoprotein | branched-chain alpha-ketoacid dehydrogenase multienzyme complex BCKDC which is regulated by phosphorylation of component E1b, residue Ser292 of the alpha-domain, the phosphorylated recombinant E1b component shows highly reduced activity compared to the nonphosphorylated one | Homo sapiens |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
2-oxoisovalerate + [dihydrolipoyllysine-residue(2-methylpropanoyl)transferase] lipoyllysine | - |
Homo sapiens | ? | - |
? | |
additional information | branched-chain alpha-ketoacid dehydrogenase multienzyme complex BCKDC which is regulated by phosphorylation of component E1b, residue Ser292 of the alpha-domain, in response to hormone and dietary stimuli, phosphorylation induces a conformational change of E1b phosphorylation loop which prevents binding of the E2b component, thiamine diphosphate and His291alpha are involved, overview | Homo sapiens | ? | - |
? | |
additional information | multistep reaction of the enzyme complex, overview | Homo sapiens | ? | - |
? |
Subunits | Comment | Organism |
---|---|---|
More | E1b component, analysis of structure-function-relationship, overview | Homo sapiens |
Synonyms | Comment | Organism |
---|---|---|
BCKDC | - |
Homo sapiens |
branched-chain alpha-ketoacid dehydrogenase multienzyme complex | - |
Homo sapiens |
More | E1b and E2b are components of the branched-chain alpha-ketoacid dehydrogenase multienzyme complex BCKDC | Homo sapiens |
Turnover Number Minimum [1/s] | Turnover Number Maximum [1/s] | Substrate | Comment | Organism | Structure |
---|---|---|---|---|---|
0.58 | - |
2-oxo-isovalerate | E1b component mutant S292Dalpha | Homo sapiens | |
2.18 | - |
2-oxo-isovalerate | E1b component mutant S302Aalpha | Homo sapiens | |
3.3 | - |
2-oxo-isovalerate | wild-type E1b component | Homo sapiens | |
3.92 | - |
2-oxo-isovalerate | E1b component mutant S292Nalpha | Homo sapiens |
Cofactor | Comment | Organism | Structure |
---|---|---|---|
thiamine diphosphate | bound to E1b component, dissociation constants of recombinant wild-type and mutant E1b components | Homo sapiens |