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A family of enzymes (transaminases, EC 2.6.1.x) that transfer an amino group from an amino acid to an α-keto acid. Glutamate oxaloacetate transaminase (EC, aspartate aminotransferase) catalyses the transfer from glutamate to oxaloacetic acid (producing aspartic acid and α-ketoglutarate) and is a pyridoxal phosphate-dependent enzyme which exists in both mitochondrial (430 aa) and cytosolic (413 aa) forms. Branched chain aminotransferases (EC, BCAT1, 393 aa; BCAT2) act on amino acids with nonlinear aliphatic side chains (leucine, isoleucine, and valine). Two clinical disorders are due to a defect of branched chain amino acid transamination, hypervalinaemia and hyperleucine–isoleucinaemia.

Subjects: Medicine and Health.

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