Any feedback?
Literature summary extracted from
- Molecular mechanism for regulation of the human mitochondrial branched-chain alpha-ketoacid dehydrogenase complex by phosphorylation (2004), Structure, 12, 2185-2196.
Protein Variants
| EC Number | Protein Variants | Comment | Organism |
|---|---|---|---|
| 1.2.4.4 | 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 |
| 1.2.4.4 | 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 |
| 1.2.4.4 | 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 |
| 1.2.4.4 | 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 |
| 1.2.4.4 | S292Q | site-directed mutagenesis, mutation of a alpha-subunit residue of complex component E1b, inactive mutant | Homo sapiens |
| 1.2.4.4 | 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]
| EC Number | KM Value [mM] | KM Value Maximum [mM] | Substrate | Comment | Organism | Structure |
|---|---|---|---|---|---|---|
| 1.2.4.4 | additional information | - |
additional information | kinetics, wild-type and mutant E1b components | Homo sapiens | |
| 1.2.4.4 | 0.028 | - |
2-oxo-isovalerate | E1b component mutant S302Aalpha | Homo sapiens |  |
| 1.2.4.4 | 0.048 | - |
2-oxo-isovalerate | wild-type E1b component | Homo sapiens | |
| 1.2.4.4 | 0.193 | - |
2-oxo-isovalerate | E1b component mutant S292Nalpha | Homo sapiens | |
| 1.2.4.4 | 4.38 | - |
2-oxo-isovalerate | E1b component mutant S292Dalpha | Homo sapiens | |
Localization
| EC Number | Localization | Comment | Organism | GeneOntology No. | Textmining |
|---|---|---|---|---|---|
| 1.2.4.4 | mitochondrion | - |
Homo sapiens | 5739 | - |
Natural Substrates/ Products (Substrates)
| EC Number | Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
|---|---|---|---|---|---|---|---|
| 1.2.4.4 | 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
| EC Number | Organism | UniProt | Comment | Textmining |
|---|---|---|---|---|
| 1.2.4.4 | Homo sapiens | - |
- |
- |
Posttranslational Modification
| EC Number | Posttranslational Modification | Comment | Organism |
|---|---|---|---|
| 1.2.4.4 | 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 and Products (Substrate)
| EC Number | Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
|---|---|---|---|---|---|---|---|
| 1.2.4.4 | 2-oxoisovalerate + [dihydrolipoyllysine-residue(2-methylpropanoyl)transferase] lipoyllysine | - |
Homo sapiens | ? | - |
? | |
| 1.2.4.4 | 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 | ? | - |
? | |
| 1.2.4.4 | additional information | multistep reaction of the enzyme complex, overview | Homo sapiens | ? | - |
? | |
Subunits
| EC Number | Subunits | Comment | Organism |
|---|---|---|---|
| 1.2.4.4 | More | E1b component, analysis of structure-function-relationship, overview | Homo sapiens |
Synonyms
| EC Number | Synonyms | Comment | Organism |
|---|---|---|---|
| 1.2.4.4 | BCKDC | - |
Homo sapiens |
| 1.2.4.4 | branched-chain alpha-ketoacid dehydrogenase multienzyme complex | - |
Homo sapiens |
| 1.2.4.4 | More | E1b and E2b are components of the branched-chain alpha-ketoacid dehydrogenase multienzyme complex BCKDC | Homo sapiens |
Turnover Number [1/s]
| EC Number | Turnover Number Minimum [1/s] | Turnover Number Maximum [1/s] | Substrate | Comment | Organism | Structure |
|---|---|---|---|---|---|---|
| 1.2.4.4 | 0.58 | - |
2-oxo-isovalerate | E1b component mutant S292Dalpha | Homo sapiens | |
| 1.2.4.4 | 2.18 | - |
2-oxo-isovalerate | E1b component mutant S302Aalpha | Homo sapiens | |
| 1.2.4.4 | 3.3 | - |
2-oxo-isovalerate | wild-type E1b component | Homo sapiens | |
| 1.2.4.4 | 3.92 | - |
2-oxo-isovalerate | E1b component mutant S292Nalpha | Homo sapiens | |
Cofactor
| EC Number | Cofactor | Comment | Organism | Structure |
|---|---|---|---|---|
| 1.2.4.4 | thiamine diphosphate | bound to E1b component, dissociation constants of recombinant wild-type and mutant E1b components | Homo sapiens | |
Use of this online version of BRENDA is free under the CC BY 4.0 license. See terms of use for full details.
Release 2023.1 (January 2023)
Legal
Curated at
Member of
