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Bckdk - branched chain ketoacid dehydrogenase kinase (house mouse)

Gene
Symbol
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).

1 Names and Identifiers

1.1 Synonyms

  • BDK
  • branched-chain alpha-ketoacid dehydrogenase kinase
  • BCKD-kinase
  • BCKDH kinase
  • BCKDHKIN
  • [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial

1.2 Other Identifiers

1.2.1 Ensembl ID

1.2.2 Alliance Gene ID

1.2.3 Bgee Gene ID

1.2.4 Enzyme Commission (EC) Number

1.2.5 MGI ID

1.2.6 VEuPathDB ID

1.2.7 Wikidata

3 Proteins

3.1 Protein 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 (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).

3.2 Protein 3D Structures

3.2.1 AlphaFold Structures

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

3.3 Protein Targets

4 BioAssays

4.1 RNAi BioAssays

5 Interactions and Pathways

5.1 Interactions

5.2 Pathways

6 Biochemical Reactions

7 Expression

8 Literature

8.1 Consolidated References

8.2 Gene-Chemical Co-Occurrences in Literature

8.3 Gene-Gene Co-Occurrences in Literature

8.4 Gene-Disease Co-Occurrences in Literature

9 Patents

9.1 Gene-Chemical Co-Occurrences in Patents

9.2 Gene-Gene Co-Occurrences in Patents

9.3 Gene-Disease Co-Occurrences in Patents

10 Classification

10.1 Gene Ontology: Biological Process

10.2 Gene Ontology: Cellular Component

10.3 Gene Ontology: Molecular Function

11 Information Sources

  1. NCBI Gene
    LICENSE
    NCBI Website and Data Usage Policies and Disclaimers
    https://www.ncbi.nlm.nih.gov/home/about/policies/
  2. PubChem
  3. Alliance of Genome Resources
    LICENSE
    All annotations and data produced by Alliance members that are accessible from alliancegenome.org are distributed under a CC BY 4.0 license (https://creativecommons.org/licenses/by/4.0/).
    https://www.alliancegenome.org/privacy-warranty-licensing
  4. BioGRID
    LICENSE
    The MIT License (MIT); Copyright Mike Tyers Lab
    https://wiki.thebiogrid.org/doku.php/terms_and_conditions
  5. STRING: functional protein association networks
  6. Mouse Genome Informatics (MGI)
    LICENSE
    MGI data and annotations are licensed under a Creative Commons Attribution 4.0 International License (CC-BY).
    https://www.informatics.jax.org/mgihome/other/copyright.shtml
  7. NCBI Gene Expression Omnibus (GEO)
  8. Swiss Institute of Bioinformatics Bgee
    LICENSE
    Creative Commons Zero license (CC0)
    https://www.bgee.org/about/
  9. UniProt
    LICENSE
    We have chosen to apply the Creative Commons Attribution (CC BY 4.0, http://creativecommons.org/licenses/by/4.0/) License to all copyrightable parts of our databases.
    https://www.uniprot.org/help/license
  10. VEuPathDB: The Eukaryotic Pathogen, Vector and Host Informatics Resource
    LICENSE
    All data on VEuPathDB websites are provided freely for public use.
    https://veupathdb.org/veupathdb/app/static-content/about.html
  11. Wikidata
  12. Gene Ontology (GO)
    LICENSE
    Gene Ontology Consortium data and data products are licensed under the Creative Commons Attribution 4.0 Unported License (https://creativecommons.org/licenses/by/4.0/legalcode)
    http://geneontology.org/docs/go-citation-policy/
  13. AlphaFold DB
    LICENSE
    All of the data provided is freely available for both academic and commercial use under Creative Commons Attribution 4.0 (CC-BY 4.0) licence terms.
    https://alphafold.ebi.ac.uk/faq
  14. Rhea - annotated reactions database
    LICENSE
    Rhea has chosen to apply the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/). This means that you are free to copy, distribute, display and make commercial use of the database in all legislations, provided you credit (cite) Rhea.
    https://www.rhea-db.org/help/license-disclaimer
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