- hyper-β-aminoisobutyric aciduria
- Elevated levels of β-aminoisobutyric acid in the urine; believed to be due to a deficiency of liver β-aminoisobutyrate:pyruvate aminotransferase.
Medical dictionary. 2011.
hyper-β-aminoisobutyric aciduria
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β-aminoisobutyrate:pyruvate aminotransferase — 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… … Medical dictionary
