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Literature summary for 2.7.11.4 extracted from

  • Samuel Zigler, J.; Hodgkinson, C.; Wright, M.; Klise, A.; Sundin, O.; Broman, K.; Hejtmancik, F.; Huang, H.; Patek, B.; Sergeev, Y.; Hose, S.; Brayton, C.; Xaiodong, J.; Vasquez, D.; Maragakis, N.; Mori, S.; Goldman, D.; Hoke, A.; Sinha, D.
    A spontaneous missense mutation in branched chain keto acid dehydrogenase kinase in the rat affects both the central and peripheral nervous systems (2016), PLoS ONE, 11, e0160447 .
    View publication on PubMedView publication on EuropePMC
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Protein Variants

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

Localization Comment Organism GeneOntology No. Textmining
mitochondrial matrix
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 Rattus norvegicus 5759
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Natural Substrates/ Products (Substrates)

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

Organism UniProt Comment Textmining
Rattus norvegicus Q00972 Sprague-Dawley
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Source Tissue

Source Tissue Comment Organism Textmining
brain
-
 Rattus norvegicus
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Substrates and Products (Substrate)

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

Synonyms Comment Organism
BCKDK
-
 Rattus norvegicus
branched chain keto acid dehydrogenase kinase
-
 Rattus norvegicus

General Information

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