Branched chain ketoacid dehydrogenase kinase (BCKDK) is highly expressed in the skeletal muscles and the gene encoding it is located on human chromosome 16p11.2.
Synonyms: Anti-BCKD-kinase; Anti-BCKDHKIN; Anti-Branched-chain alpha-ketoacid dehydrogenase kinase; Anti-[3-Methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial precursor
Storage: -20C
Application: All Prestige Antibodies Powered by Atlas Antibodies are developed and validated by the Human Protein Atlas (HPA) project (www.proteinatlas.org)and as a result, are supported by the most extensive characterization in the industry. The Human Protein Atlas project can be subdivided into three efforts: Human Tissue Atlas, Cancer Atlas, and Human Cell Atlas. The antibodies that have been generated in support of the Tissue and Cancer Atlas projects have been tested by immunohistochemistry against hundreds of normal and disease tissues and through the recent efforts of the Human Cell Atlas project, many have been characterized by immunofluorescence to map the human proteome not only at the tissue level but now at the subcellular level. These images and the collection of this vast data set can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. To view these protocols and other useful information about Prestige Antibodies and the HPA, visit sigma.com/prestige.
Biochem Physiol Actions: Branched chain ketoacid dehydrogenase kinase (BCKDK) plays an important role as a negative regulator of the branched-chain a-keto acid dehydrogenase complex (BCKD). It reversibly phosphorylates the subunits of BCKD and inhibits its function. This leads to a decrease in the catabolism of ketoacids of amino acids like leucine and isoleucine. Mutations in the gene encoding BCKDK are linked to neurobehavioral disorder in a form of autism.
Legal Information: Prestige Antibodies is a registered trademark of Sigma-Aldrich Co. LLC