beta-aminoisobutyrate:pyruvate aminotransferase

β-a·mi·no·i·so·bu·ty·rate:py·ru·vate a·mi·no·trans·fer·ase

(ă-mē'nō-ī'sō-bū'ti-rāt pī-rū'vāt ă-mē'nō-tranz'fĕr-ās), β-Aminosiobutyrate:pyruvate transaminase; an enzyme that catalyzes the reversible transfer of an amino group from β-aminoisobutyrate to pyruvate, producing l-alanine and methylmalonate semialdehyde; a step in valine degradation. A deficiency of this enzyme results in hyper-β-aminoisobutyric aciduria.