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3-methylglutaconyl-CoA hydratase

Pronunciation: meth'il-glu'ta-kon'il hi'dra-tas

Definition: An enzyme that catalyzes the reaction of trans-3-methylglutaconyl-CoA and water to form 3-hydroxy-3-methylglutaconyl-CoA; this enzyme participates in the pathway for l-leucine degradation; a deficiency of this enzyme will result in 3-methylglutaconic aciduria.

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