| Tissue Specificity | Highest expression in liver. Expressed in pancreas, kidney, intestine, testis, fibroblasts and lymphoblasts. Very low expression in brain and skeletal muscle. The relative expression of isoform 2 (at mRNA level) is highest in heart (30%), skeletal muscle (22%), and brain (14%). |
| Function | Mitochondrial 3-hydroxy-3-methylglutaryl-CoA lyase that catalyzes a cation-dependent cleavage of (S)-3-hydroxy-3-methylglutaryl-CoA into acetyl-CoA and acetoacetate, a key step in ketogenesis. Terminal step in leucine catabolism. Ketone bodies (beta-hydroxybutyrate, acetoacetate and acetone) are essential as an alternative source of energy to glucose, as lipid precursors and as regulators of metabolism. |
| Protein Name | Hydroxymethylglutaryl-Coa Lyase - MitochondrialHlHmg-Coa Lyase3-Hydroxy-3-Methylglutarate-Coa Lyase |
| Database Links | Reactome: R-HSA-77111Reactome: R-HSA-9033241 |
| Cellular Localisation | Mitochondrion MatrixPeroxisomeUnprocessed Form Is Peroxisomal |
| Alternative Antibody Names | Anti-Hydroxymethylglutaryl-Coa Lyase - Mitochondrial antibodyAnti-Hl antibodyAnti-Hmg-Coa Lyase antibodyAnti-3-Hydroxy-3-Methylglutarate-Coa Lyase antibodyAnti-HMGCL antibody |
Information sourced from Uniprot.org
