Bckdk - branched chain ketoacid dehydrogenase kinase (house mouse)
Gene
Symbol
Taxonomy
Dates
- Create:2016-09-14
- Modify:2025-01-28
Description
Enables protein kinase activity. Predicted to be involved in several processes, including branched-chain amino acid catabolic process; regulation of small molecule metabolic process; and spermatogenesis. Located in mitochondrion. Is expressed in several structures, including genitourinary system; heart; liver; lung; and skeletal muscle. Used to study autism spectrum disorder and branched-chain keto acid dehydrogenase kinase deficiency. Human ortholog(s) of this gene implicated in branched-chain keto acid dehydrogenase kinase deficiency. Orthologous to human BCKDK (branched chain keto acid dehydrogenase kinase).
- BDK
- branched-chain alpha-ketoacid dehydrogenase kinase
- BCKD-kinase
- BCKDH kinase
- BCKDHKIN
- [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial
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 (By similarity). 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-334, leading to conformational changes that interrupt substrate channeling between E1 and E2 and inactivates the BCKDH complex (By similarity). 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 (By similarity). Recognizes phosphosites having SxxE/D canonical motif (By similarity).
Highly accurate protein structure prediction with AlphaFold. Nature. 2021 Aug;596(7873):583-589. DOI:10.1038/s41586-021-03819-2. PMID:34265844; PMCID:PMC8371605
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