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Literature summary extracted from

  • Machius, M.; Wynn, R.M.; Chuang, J.L.; Li, J.; Kluger, R.; Yu, D.; Tomchick, D.R.; Brautigam, C.A.; Chuang, D.T.
    A versatile conformational switch regulates reactivity in human branched-chain alpha-ketoacid dehydrogenase (2006), Structure, 14, 287-298.
    View publication on PubMed

Crystallization (Commentary)

EC Number Crystallization (Comment) Organism
1.2.4.4 crystals of E1b grown in the absence and presence of substrates at 22ΒΊC via the vapor-diffusion method, key tyrosine residue in the E1b active site, functions as a conformational switch to reduce the reactivity of the thiamin diphosphate cofactor, the tyrosine switch further remodels an E1b loop region to promote E1b binding to E2b Homo sapiens

Protein Variants

EC Number Protein Variants Comment Organism
1.2.4.4 additional information hE1b-Tyr113Phe mutant, specifically affects the decarboxylation reaction, thiamine diphosphate appears hyperactive, is prone to paracatalytic inactivation Homo sapiens

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
1.2.4.4 alpha-keto-beta-methylvaleric acid + NADH Homo sapiens
-
?
-
?
1.2.4.4 alpha-ketoisocaproic acid + NADH Homo sapiens
-
?
-
?
1.2.4.4 alpha-ketoisovaleric acid + NADH Homo sapiens
-
?
-
?

Organism

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

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
1.2.4.4 alpha-keto-beta-methylvaleric acid + NADH
-
Homo sapiens ?
-
?
1.2.4.4 alpha-ketoisocaproic acid + NADH
-
Homo sapiens ?
-
?
1.2.4.4 alpha-ketoisovaleric acid + NADH
-
Homo sapiens ?
-
?

Synonyms

EC Number Synonyms Comment Organism
1.2.4.4 BCKDC
-
Homo sapiens
1.2.4.4 branched-chain alpha-ketoacid dehydrogenase complex
-
Homo sapiens
1.2.4.4 E1b
-
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 0.1983
-
alpha-ketoisovaleric acid wild-type Homo sapiens
1.2.4.4 2.85
-
alpha-ketoisovaleric acid hE1b-Tyr113Phe mutant Homo sapiens

Cofactor

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