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2-methylacetoacetyl-CoA thiolase

Pronunciation: meth'il-a-se'to-a'se-til thi'o-las

Definition: An enzyme that is part of the l-isoleucine degradation pathway; it catalyzes the conversion of 2-methylacetoacetyl-CoA to acetyl-CoA and propionyl-CoA. A deficiency of this enzyme leads to an accumulation of 2-methylacetoacetyl-CoA, causing episodes of severe metabolic acidosis and ketosis.

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