Results for "Tris(Hydroxymethyl)Aminomethane": 2 genes

Name

HMGCL Gene

3-hydroxymethyl-3-methylglutaryl-CoA lyase

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, Oct 2009]

HMGCLL1 Gene

3-hydroxymethyl-3-methylglutaryl-CoA lyase-like 1

Enables hydroxymethylglutaryl-CoA lyase activity. Involved in ketone body biosynthetic process. Located in several cellular components, including cytosol; endoplasmic reticulum; and perinuclear region of cytoplasm. [provided by Alliance of Genome Resources, Mar 2025]