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Literature summary extracted from

  • Brosnan, J.T.; Brosnan, M.E.
    Branched-chain amino acids: enzyme and substrate regulation (2006), J. Nutr., 136, 207S-211S.
    View publication on PubMed

Activating Compound

EC Number Activating Compound Comment Organism Structure
1.2.4.4 additional information activated by dephosphorylation Homo sapiens

Application

EC Number Application Comment Organism
1.2.4.4 additional information regulation of BCKD kinase expression by nutritional, hormonal, and pathological factors Homo sapiens

Cloned(Commentary)

EC Number Cloned (Comment) Organism
1.2.4.4
-
 Homo sapiens

Inhibitors

EC Number Inhibitors Comment Organism Structure
1.2.4.4 alpha-Ketoisocaproate allosteric inhibition, also by other branched-chain keto acids, although they are less effective as compared to alpha-ketoisocaproate Homo sapiens
1.2.4.4 additional information inhibited by phosphorylation, allosteric inhibition by CoA esters that arise during branched-chain amino acid catabolism Homo sapiens
1.2.4.4 NADH active dephosphorylated BCKD, allosteric inhibition Homo sapiens
2.6.1.42 gabapentin inhibitor of the cytosolic enzyme Homo sapiens

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
2.6.1.42 cytosol
-
 Homo sapiens 5829
-
2.6.1.42 mitochondrion
-
 Homo sapiens 5739
-

Organism

EC Number Organism UniProt Comment Textmining
1.2.4.4 Homo sapiens
-
-
-
2.6.1.42 Homo sapiens
-
-
-

Source Tissue

EC Number Source Tissue Comment Organism Textmining
1.2.4.4 adipose tissue
-
 Homo sapiens
-
1.2.4.4 brain
-
 Homo sapiens
-
1.2.4.4 heart lowest activity Homo sapiens
-
1.2.4.4 intestine
-
 Homo sapiens
-
1.2.4.4 kidney
-
 Homo sapiens
-
1.2.4.4 liver
-
 Homo sapiens
-
1.2.4.4 skeletal muscle highest activity Homo sapiens
-
2.6.1.42 glial cell cytosolic enzyme form Homo sapiens
-
2.6.1.42 neuron mitochondrial enzyme form Homo sapiens
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
1.2.4.4 additional information flux-generating step for branched-chain amino acid catabolism Homo sapiens ?
-
 ?
2.6.1.42 L-leucine + 2-oxoglutarate
-
 Homo sapiens 4-methyl-2-oxopentanoate + L-glutamate
-
 ?
2.6.1.42 L-valine + 2-oxoglutarate
-
 Homo sapiens 3-methyl-2-oxobutanoate + L-glutamate
-
 ?

Synonyms

EC Number Synonyms Comment Organism
1.2.4.4 BCKD
-
 Homo sapiens
1.2.4.4 branched-chain alpha-keto acid dehydrogenase
-
 Homo sapiens
2.6.1.42 BCAA aminotransferase
-
 Homo sapiens
2.6.1.42 BcaT
-
 Homo sapiens

Cofactor

EC Number Cofactor Comment Organism Structure
2.6.1.42 pyridoxal 5'-phosphate
-
 Homo sapiens