3-hydroxy-3-methylglutaryl-coa lyase deficiency

Catalysis of the reaction: 3-hydroxy-3-(4-methylpent-3-en-1-yl)glutaryl-CoA + 4 H(+) = 7-methyl-3-oxooct-6-enoyl-CoA + acetate.

Disease Alternative Name

3-hydroxy-3-isohexenylglutaryl-coa isopentenylacetoacetyl-coa-lyase activity
3-hydroxy-3-(4-methylpent-3-en-1-yl)glutaryl-coa acetate-lyase (7-methyl-3-oxooct-6-enoyl-coa-forming)
hydroxyisohexenylglutaryl-coa:acetatelyase activity
beta-hydroxy-beta-isohexenylglutaryl coa-lyase activity
3-hydroxy-3-(4-methylpent-3-en-1-yl)glutaryl-coa acetate-lyase activity
3-hydroxy-3-isohexenylglutaryl coenzyme a lyase activity
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