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EC Number General Information Commentary Reference
Display the word mapDisplay the reaction diagram Show all sequences 2.7.11.4malfunction patients with homozygous BCKDK mutations display reductions in BCKDK messenger RNA and protein, E1alpha phosphorylation, and plasma branched-chain amino acids. Inactivating mutations in the gene BCKDK in consanguineous families are associated with autism, epilepsy, and intellectual disability, geno- and phenotyping, overview 723772
Display the word mapDisplay the reaction diagram Show all sequences 2.7.11.4malfunction the BDK activity is decreased in the livers of streptozotocin-induced diabetic rats 721483
Display the word mapDisplay the reaction diagram Show all sequences 2.7.11.4malfunction the endurance performance enhanced by 2 weeks of running training is somewhat, but significantly less in BDK-mKO mice than in control mice. Skeletal muscle of BDK-mKO mice has low levels of glycogen. Catabolism of branched-chain amino acids (BCAAs) is greatly enhanced in the muscle of BDK-mKO mice and produces branched-chain acyl-carnitine, which induces perturbation of energy metabolism in the muscle 762226
Display the word mapDisplay the reaction diagram Show all sequences 2.7.11.4metabolism the enzyme is involved in the regulation of the branched-chain alpha-keto acid dehydrogenase complex by inactivating it through phopshorylation, complex regulation and effects of the drug benzofibrate, overview 723788
Display the word mapDisplay the reaction diagram Show all sequences 2.7.11.4more activity of BDK practically corresponds with plasma insulin concentrations 721483
Display the word mapDisplay the reaction diagram Show all sequences 2.7.11.4physiological function BCKDk contains multiple epitopes that can induce myocarditis. The BCKDk 111-130 is the primary epitope that induces both autoimmune myocarditis and hepatitis in A/J mice. The disease induced by BCKDk peptides can serve as a useful model to study the autoimmune events of inflammatory heart and liver diseases 761232
Display the word mapDisplay the reaction diagram Show all sequences 2.7.11.4physiological function branched-chain alpha-keto acid dehydrogenase kinase is responsible for the regulation of branched-chain alpha-keto acid dehydrogenase complex, which is the rate-limiting enzyme in the catabolism of branched-chain amino acids 721483
Display the word mapDisplay the reaction diagram Show all sequences 2.7.11.4physiological function rate-limiting enzyme in branched-chain amino acids catabolism 760972
Display the word mapDisplay the reaction diagram Show all sequences 2.7.11.4physiological function rate-limiting enzyme in branched-chain amino acids catabolism, which plays an important role in many serious human diseases. The enzyme promotes cell transformation or colorectal cancer by enhancing the MAPK signaling pathway through direct mitogen-activated protein kinase kinase (MEK) phosphorylation, rather than by branched-chain amino acids catabolism 760972
Display the word mapDisplay the reaction diagram Show all sequences 2.7.11.4physiological function the enzyme (Bckdk) is an integral part of an enzyme complex located in the mitochondrial matrix of many tissues which regulates the levels of the branched-chain amino acids (BCAAs), leucine, isoleucine and valine 762211
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