Protein Variants | Comment | Organism |
---|---|---|
additional information | circulating levels of the branched-chain amino acids (BCAAs) are reduced by 70-80% in animals homozygous for the mutation causing a phenotype named frogleg. The mutationis located within the kinase domain of Bckdk. The frogleg phenotype shares important characteristics with a previously described Bckdk knockout mouse and with human subjects with Bckdk mutations. The mutation affects both the central and peripheral nervous systems | Rattus norvegicus |
Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
mitochondrial matrix | - |
Rattus norvegicus | 5759 | - |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
ATP + [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] | Rattus norvegicus | the enzyme (BCKDK) phosphorylates Ser293 of the E1alpha subunit of the BCKDH protein, which catalyzes the rate-limiting step in the catabolism of the branched-chain amino acids (BCAAs), inhibiting 3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring) (BCKDH) and thereby, limiting breakdown of the BCAAs | ADP + [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] phosphate | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Rattus norvegicus | Q00972 | Sprague-Dawley | - |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
brain | - |
Rattus norvegicus | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
ATP + [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] | - |
Rattus norvegicus | ADP + [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] phosphate | - |
? | |
ATP + [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] | the enzyme (BCKDK) phosphorylates Ser293 of the E1alpha subunit of the BCKDH protein, which catalyzes the rate-limiting step in the catabolism of the branched-chain amino acids (BCAAs), inhibiting 3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring) (BCKDH) and thereby, limiting breakdown of the BCAAs | Rattus norvegicus | ADP + [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] phosphate | - |
? |
Synonyms | Comment | Organism |
---|---|---|
BCKDK | - |
Rattus norvegicus |
branched chain keto acid dehydrogenase kinase | - |
Rattus norvegicus |
General Information | Comment | Organism |
---|---|---|
physiological 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 | Rattus norvegicus |