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β-aminoisobutyrate:pyruvate aminotransferase

Pronunciation: ă-mē′nō-ī′sō-bū′ti-rāt pī-rū′vāt ă-mē′nō-tranz′fĕr-ās

Definition: 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.

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© Copyright 2018 Wolters Kluwer. All Rights Reserved. Review date: Sep 19, 2016.