Literature associating BCKDK and branched-chain keto acid dehydrogenase kinase deficiency
BCKDK [ENSP00000378405]
[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial; 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.
Synonyms: BCKDK, A0A024QZA9, H3BNP3, H3BQP2, H3BS02 ...