|Group: Atypical Family: PDHK Subfamily: BCKDK|
Description: branched chain ketoacid dehydrogenase kinase
Gene Symbol: BCKDK
UniprotKB/SwissProt: BCKD_HUMAN (O14874)
Function: 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 (By similarity).
Subcellular Localization: Mitochondrion matrix.