α-methylacetoaceticaciduria

α-methylacetoaceticaciduria
α-meth·yl·ac·e·to·ace·tic·ac·id·uria (meth″əl-ə-se″to-ə-se″tik-as″ĭ-duґre-ə) an autosomal recessive aminoacidopathy due to deficiency of α-methylacetoacetyl CoA thiolase, the β-ketothiolase catalyzing the final step in isoleucine catabolism; it is characterized by episodes of severe metabolic ketoacidosis and urinary excretion of several intermediates of isoleucine catabolism, including α-methylacetoacetic acid.

Medical dictionary. 2011.

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