3-@hydroxyisobutyryl-coa hydrolase deficiency

Catalysis of the reaction: 3-hydroxy-2-methylpropanoyl-CoA + H2O = CoA + 3-hydroxy-2-methylpropanoate.

Disease Alternative Name

3-hydroxy-isobutyryl coa hydrolase activity
3-hydroxy-2-methylpropanoyl-coa hydrolase activity
hib coa deacylase activity
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