[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial

Definition:

Catalyzes the phosphorylation and inactivation of the branched-chain alpha-ketoacid dehydrogenase complex, the key regulatory enzyme of the valine, leucine and isoleucine catabolic pathways. Key enzyme that regulate the activity state of the BCKD complex.

References:

[1]. Angels García-Cazorla, et al. Two novel mutations in the BCKDK (branched-chain keto-acid dehydrogenase kinase) gene are responsible for a neurobehavioral deficit in two pediatric unrelated patients. Hum Mutat. 2014 Apr;35(4):470-7. [Content Brief]

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