hyper-β-aminoisobutyric aciduria

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.

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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

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