HMGCL (3-hydroxy-3-methylglutaryl-Coenzyme A (CoA) lyase), also known as HMG-CoA lyase or HL, is a mitochondrial matrix protein that belongs to the HMG-CoA lyase family of proteins. Expressed in liver, lymphoblasts and fibroblasts, HMGCL exists as a homodimer and participates in leucine catabolism and ketogenesis, the hepatic synthesis of ketone bodies that, during fasting, provide a major source of energy for heart, brain and kidney. More specifically, HMGCL catalyzes the final step of these processes, the cleavage of 3-hydroxy-3-methylglutaryl CoA to acetoacetic acid and acetyl CoA. Mutations in the gene encoding HMGCL can lead to HMG-CoA lyase deficiency (also known as HL deficiency or hydroxymethylglutaricaciduria), a metabolic disease that, if left untreated, results in hypoglycemia and coma.
Key Feature
Clonality
Polyclonal
Isotype
IgG
Host Species
Rabbit
Tested Applications
WBIPELISA
recommended for detection of HMGCL of mouse, rat, human and canine origin by WB, IP and ELISA: