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β-alanine–pyruvate aminotransferase β-alanine–pyruvate transaminase.
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β-alanine-pyruvate transaminase [EC 2.6.1.18] a mitochondrial enzyme of the transferase class that catalyzes the transfer of the amino group from β-alanine to pyruvate, forming alanine and malonate semialdehyde. The enzyme can also act on R-β-aminoisobutyrate.
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β-alaninemia hyper-β-alaninemia.
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β-aminoisobutyrate–pyruvate transaminase an enzyme of the transferase class that catalyzes the oxidative deamination of R-β-aminoisobutyrate, transferring the amino group to pyruvate; the reaction is a step in the catabolism of thymine. Deficiency of the enzyme, an autosomal recessive trait, causes β-aminoisobutyricaciduria. In EC nomenclature, called (R)-3-amino-2-methylpropionate–pyruvate transaminase. Called also β-aminoisobutyrate–pyruvate aminotransferase.
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β-aminoisobutyricaciduria excessive excretion of R-β-aminoisobutyric acid in the urine, occurring as a benign metabolic variant due to deficiency of β-aminoisobutyrate–pyruvate transaminase; it also occurs in certain illnesses in which rapid tissue destruction and deoxyribonucleic acid catabolism occur. Called also hyper-β-aminoisobutyricaciduria.
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