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Literature summary extracted from

  • Li, J.; Machius, M.; Chuang, J.L.; Wynn, R.M.; Chuang, D.T.
    The two active sites in human branched-chain alpha-keto acid dehydrogenase operate independently without an obligatory alternating-site mechanism (2007), J. Biol. Chem., 282, 11904-11913.
    View publication on PubMed

Cloned(Commentary)

EC Number Cloned (Comment) Organism
1.2.4.4 expressed in Escherichia coli BL-21 cells Homo sapiens

Crystallization (Commentary)

EC Number Crystallization (Comment) Organism
1.2.4.4
-
 Homo sapiens

Protein Variants

EC Number Protein Variants Comment Organism
1.2.4.4 D108N less thermostable than wild type enzyme Homo sapiens
1.2.4.4 D200A less thermostable than wild type enzyme Homo sapiens
1.2.4.4 D200A/D108N the mutant is defective in folding and assembly and as no detectable overall activity Homo sapiens
1.2.4.4 D200N less thermostable than wild type enzyme Homo sapiens
1.2.4.4 E198Q/D200N the mutant is defective in folding and assembly and as no detectable overall activity Homo sapiens
1.2.4.4 additional information the hybrid E1b heterotetramer carrying one wild type, one S292Qalpha subunit, and two wild type beta subunits contains only one functional active site and exhibits nearly 40% of the wild type activity Homo sapiens
1.2.4.4 S292Q no activity Homo sapiens

Inhibitors

EC Number Inhibitors Comment Organism Structure
1.2.4.4 additional information branched-chain alpha-keto acid dehydrogenase overall activity is nullified at 50% phosphorylation of E1b Homo sapiens

KM Value [mM]

EC Number KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
1.2.4.4 0.119
-
 2-oxo-isovalerate wild type enzyme Homo sapiens

Localization

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

Organism

EC Number Organism UniProt Comment Textmining
1.2.4.4 Homo sapiens
-
-
-

Purification (Commentary)

EC Number Purification (Comment) Organism
1.2.4.4 His Trap HP column chromatography and Strep-Tactin Superflow gravity column chromatography Homo sapiens

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
1.2.4.4 2-oxoisovalerate + 2,6-dichlorophenolindophenol + CoA
-
 Homo sapiens ? + CO2 + reduced 2,6-dichlorophenolindophenol
-
 ?

Subunits

EC Number Subunits Comment Organism
1.2.4.4 heterotetramer x-ray crystallography Homo sapiens

Synonyms

EC Number Synonyms Comment Organism
1.2.4.4 BCKDC
-
 Homo sapiens
1.2.4.4 branched-chain alpha-keto acid dehydrogenase
-
 Homo sapiens
1.2.4.4 E1b component
-
 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 9.4
-
 2-oxo-isovalerate wild type enzyme Homo sapiens

Cofactor

EC Number Cofactor Comment Organism Structure
1.2.4.4 thiamine diphosphate dependent Homo sapiens