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Literature summary for 1.2.4.4 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)

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

Crystallization (Commentary)

Crystallization (Comment) Organism
-
Homo sapiens

Protein Variants

Protein Variants Comment Organism
D108N less thermostable than wild type enzyme Homo sapiens
D200A less thermostable than wild type enzyme Homo sapiens
D200A/D108N the mutant is defective in folding and assembly and as no detectable overall activity Homo sapiens
D200N less thermostable than wild type enzyme Homo sapiens
E198Q/D200N the mutant is defective in folding and assembly and as no detectable overall activity Homo sapiens
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
S292Q no activity Homo sapiens

Inhibitors

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

KM Value [mM]

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

Localization

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

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Purification (Commentary)

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

Substrates and Products (Substrate)

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

Subunits

Subunits Comment Organism
heterotetramer x-ray crystallography Homo sapiens

Synonyms

Synonyms Comment Organism
BCKDC
-
Homo sapiens
branched-chain alpha-keto acid dehydrogenase
-
Homo sapiens
E1b component
-
Homo sapiens

Turnover Number [1/s]

Turnover Number Minimum [1/s] Turnover Number Maximum [1/s] Substrate Comment Organism Structure
9.4
-
2-oxo-isovalerate wild type enzyme Homo sapiens

Cofactor

Cofactor Comment Organism Structure
thiamine diphosphate dependent Homo sapiens