α-methylacetoacetyl CoA thiolase

α-meth·yl·ac·e·to·ac·e·tyl CoA thi·o·lase (meth″əl-ə-se″to-asґə-tēl ko-aґ thiґo-lās) an acetyl-CoA C-acyltransferase (β-ketothiolase) specifically catalyzing the final step in isoleucine catabolism, the cleavage of α-methylacetoacetyl CoA to form acetyl CoA and propionyl CoA. Deficiency of the enzyme, an autosomal recessive trait, results in α-methylacetoaceticaciduria.