Tissue Specificity | Ubiquitous. |
Post Translational Modifications | Autophosphorylated. |
Function | Serine/threonine-protein kinase component of macronutrients metabolism. Forms a functional kinase and phosphatase pair with PPM1K, serving as a metabolic regulatory node that coordinates branched-chain amino acids (BCAAs) with glucose and lipid metabolism via two distinct phosphoprotein targets: mitochondrial BCKDHA subunit of the branched-chain alpha-ketoacid dehydrogenase (BCKDH) complex and cytosolic ACLY, a lipogenic enzyme of Krebs cycle. Phosphorylates and inactivates mitochondrial BCKDH complex a multisubunit complex consisting of three multimeric components each involved in different steps of BCAA catabolism: E1 composed of BCKDHA and BCKDHB, E2 core composed of DBT monomers, and E3 composed of DLD monomers. Associates with the E2 component of BCKDH complex and phosphorylates BCKDHA on Ser-337, leading to conformational changes that interrupt substrate channeling between E1 and E2 and inactivates the BCKDH complex. Phosphorylates ACLY on Ser-455 in response to changes in cellular carbohydrate abundance such as occurs during fasting to feeding metabolic transition. Refeeding stimulates MLXIPL/ChREBP transcription factor, leading to increased BCKDK to PPM1K expression ratio, phosphorylation and activation of ACLY that ultimately results in the generation of malonyl-CoA and oxaloacetate immediate substrates of de novo lipogenesis and glucogenesis, respectively. Recognizes phosphosites having SxxE/D canonical motif. |
Peptide Name | Branched-Chain Alpha-Ketoacid Dehydrogenase KinaseBckdh KinaseBckdhkinBdk3-Methyl-2-Oxobutanoate Dehydrogenase Lipoamide Kinase - Mitochondrial |
Database Links | Reactome: R-HSA-70895 |
Cellular Localisation | Mitochondrion MatrixDetected In The Cytosolic Compartment Of Liver Cells |
Alternative Peptide Names | Branched-Chain Alpha-Ketoacid Dehydrogenase Kinase proteinBckdh Kinase proteinBckdhkin proteinBdk protein3-Methyl-2-Oxobutanoate Dehydrogenase Lipoamide Kinase - Mitochondrial proteinBCKDK protein |
Information sourced from Uniprot.org