The protein encoded by this gene belongs to the HMG-CoA
lyase family. It is a mitochondrial enzyme that catalyzes the final
step of leucine degradation and plays a key role in ketone body
formation. Mutations in this gene are associated with HMG-CoA lyase
deficiency. Alternatively spliced transcript variants encoding
different isoforms have been found for this gene. [provided by
RefSeq].
