(HIBCH) 3-Hydroxyisobutryl-CoA Hydrolase Deficiency

Beta-hydroxyisobutyryl-CoA hydrolase is responsible for the specific hydrolysis of HIBYL-CoA, a valine catabolite, as well as the hydrolysis of beta-hydroxypropionyl-CoA, an intermediate in a minor pathway of propionate metabolism.

Informative Websites

3-Hydroxyisobutyrl-CoA Hydrolase Deficiency – Pub Med
Deficiency of Beta-Hydroxyisobutryl CoA Deacylase – Pub Med
Primary structure and tissue-specific expression of human beta-hydroxyisobutyryl-coenzyme A hydrolase – Pub Med

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