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Medical Definition of Beta-aminoisobutyrate:pyruvate aminotransferase
1. Beta-aminosiobutyrate:pyruvate transaminase;an enzyme that catalyses the reversible transfer of an amino group from beta-aminoisobutyrate to pyruvate, producing l-alanine and methylmalonate semialdehyde. A step in valine degradation. A deficiency of beta-aminoisobutyrate:pyruvate aminotransferase results in hyper-beta-aminoisobutyric aciduria. (05 Mar 2000)