β-aminoisobutyrate:pyruvate aminotransferase

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 this enzyme results in hyper-β-aminoisobutyric aciduria.

Medical dictionary. 2011.

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  • (R)-3-amino-2-methylpropionate-pyruvate transaminase — In enzymology, a (R) 3 amino 2 methylpropionate pyruvate transaminase (EC number|2.6.1.40) is an enzyme that catalyzes the chemical reaction:(R) 3 amino 2 methylpropanoate + pyruvate ightleftharpoons 2 methyl 3 oxopropanoate + L alanineThus, the… …   Wikipedia

  • β-aminoisobutyrate–pyruvate transaminase — β ami·no·iso·bu·ty·rate–py·ru·vate trans·am·i·nase (ə me″no i″so buґtər āt piґroo vāt trans amґĭ nās) an enzyme of the transferase class that catalyzes the oxidative deamination of R β… …   Medical dictionary

  • 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

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