Any feedback?
Please rate this page
(literature.php)
(0/150)

BRENDA support

Literature summary for 1.2.4.4 extracted from

  • Wynn, R.M.; Kato, M.; Machius, M.; Chuang, J.L.; Li, J.; Tomchick, D.R.; Chuang, D.T.
    Molecular mechanism for regulation of the human mitochondrial branched-chain alpha-ketoacid dehydrogenase complex by phosphorylation (2004), Structure, 12, 2185-2196.
    View publication on PubMed

Protein Variants

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 [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

Localization Comment Organism GeneOntology No. Textmining
mitochondrion
-
Homo sapiens 5739
-

Natural Substrates/ Products (Substrates)

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

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Posttranslational Modification

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 and Products (Substrate)

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

Subunits Comment Organism
More E1b component, analysis of structure-function-relationship, overview Homo sapiens

Synonyms

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 [1/s]

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

Cofactor Comment Organism Structure
thiamine diphosphate bound to E1b component, dissociation constants of recombinant wild-type and mutant E1b components Homo sapiens