Center for Bioinformatics
Oxidoreductases | Transferases | Hydrolases | Lyases | Isomerases | Ligases

Basic Information

Enzyme Number

2.7.11.4

Official Name

[3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)]

Name from literature

branched chain ketoacid dehydrogenase kinase

Pathway from literature

branched-chain amino acid catabolism

Organisms

Rat (10116)

Genome localization

1q36[29603 ],

Comments

The enzyme has no activating compound but is specific for its substrate. It is a mitochondrial enzyme associated with the branched-chain 2-oxoacid dehydrogenase complex. Phosphorylation inactivates EC 1.2.4.4, 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring).

Rate-limiting Description

"Since the E1 component is rate-limiting for activity and also the component of the complex inhibited by phosphorylation, this decrease in number further limits overall enzyme activity and makes the complex more sensitive to regulation by phosphorylation in this nutritional state." (9381974)

"The branched-chain alpha-ketoacid dehydrogenase complex, catalyst for the rate-limiting step of branched-chain amino acid catabolism, is controlled by a highly specific protein kinase (branched-chain alpha-ketoacid dehydrogenase kinase) that associates tightly with the complex." (7840610)

Regulatory Information

Regulatory type

Detail

phosphorylation;

Q00972:from_uniprot:31_Phosphoserine

phosphorylation;

Q00972:from_uniprot:33_Phosphoserine

phosphorylation;

Q00972:from_uniprot:35_Phosphothreonine

Gene ontology

Gene ontology

GO:0005947 (C) mitochondrial alpha-ketoglutarate dehydroge... [Q00972 ];
GO:0047323 (F) [3-methyl-2-oxobutanoate dehydrogenase (ace... [Q00972 ];
GO:0009083 (P) branched chain family amino acid catabolic ... [Q00972 ];
GO:0007165 (P) signal transduction [Q00972 ];
GO:0018106 (P) peptidyl-histidine phosphorylation [Q00972 ];
GO:0005524 (F) ATP binding [Q00972 ];
GO:0004674 (F) protein serine/threonine kinase activity [Q00972 ];
GO:0000155 (F) two-component sensor activity [Q00972 ];

Subcellular localization

Localization

mitochondrion [Q00972 ];

Links

SwissProt

Q00972

Entrez Gene

29603



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  Last Modified: 2009-03-24  
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