Gene Symbol | BCKDK |
---|---|
Entrez Gene | 10295 |
Alt Symbol | BCKDKD, BDK |
Species | Human |
Gene Type | protein-coding |
Description | branched chain ketoacid dehydrogenase kinase |
Other Description | BCKD-kinase|BCKDHKIN|[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial|branched chain alpha-ketoacid dehydrogenase kinase |
Swissprots | Q96G95 A8MY43 Q96IN5 Q6FGL4 O14874 |
Accessions | EAW52160 EAW52161 EAW52162 EAW52163 EAW52164 O14874 AF026548 AAB82714 AK130145 AK294691 BAG57850 BC007363 AAH07363 BC009872 AAH09872 BM679327 BX427330 CR542093 CAG46890 DQ893687 ABM84613 DQ896864 ABM87863 NM_001122957 NP_001116429 NM_001271926 NP_001258855 NM_005881 NP_005872 |
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. {ECO:0000269|PubMed:24449431}. |
Subcellular Location | Mitochondrion matrix. Mitochondrion {ECO:0000269|PubMed:24449431}. |
Tissue Specificity | Ubiquitous. |