3-hydroxy-3-methylglutaryl-CoA lyase

3-hydroxy-3-methylglutaryl-CoA lyase (or HMG-CoA lyase) plays an essential role in breaking down dietary proteins and fats for energy.

Specifically, the enzyme is responsible for processing leucine, an amino acid that is part of many proteins.



Pathology
Mutations in the HMGCL gene cause 3-hydroxy-3-methylglutaryl-CoA lyase deficiency.