Enables carboxylic acid binding activity; fatty-acyl-CoA binding activity; and hydroxymethylglutaryl-CoA lyase activity. Involved in several processes, including acyl-CoA metabolic process; ketone body biosynthetic process; and liver development. Predicted to be located in peroxisome. Predicted to be part of protein-containing complex. Predicted to be active in mitochondrion. Human ortholog(s) of this gene implicated in amino acid metabolic disorder. Orthologous to human HMGCL (3-hydroxy-3-methylglutaryl-CoA lyase); PARTICIPATES IN 3-hydroxy-3-methylglutaryl-CoA lyase deficiency pathway; 3-hydroxyisobutyric aciduria pathway; 3-methylcrotonyl CoA carboxylase 1 deficiency pathway; INTERACTS WITH 2,3,7,8-tetrachlorodibenzodioxine; 6-propyl-2-thiouracil; acetamide.
[Clofibrate co-treated with Acetaminophen] affects the expression of HMGCL mRNA and PPARA affects the reaction [[Clofibrate co-treated with Acetaminophen] affects the expression of HMGCL mRNA]
[Clofibrate co-treated with Acetaminophen] affects the expression of HMGCL mRNA and PPARA affects the reaction [[Clofibrate co-treated with Acetaminophen] affects the expression of HMGCL mRNA]
[perfluorooctane sulfonic acid co-treated with Cellulose] results in increased expression of HMGCL mRNA and [perfluorooctane sulfonic acid co-treated with Inulin] results in increased expression of HMGCL mRNA
Mitochondrial 3-hydroxy-3-methylglutaryl CoA synthase and carnitine palmitoyltransferase II are potential control sites of hepatic ketogenesis under conditions of peroxisome proliferation.
Molecular cloning of rat mitochondrial 3-hydroxy-3-methylglutaryl-CoA lyase and detection of the corresponding mRNA and of those encoding the remaining enzymes comprising the ketogenic 3-hydroxy-3-methylglutaryl-CoA cycle in central nervous system of suckling rat.
3-Hydroxy-3-methylglutaryl coenzyme A lyase (HL). Cloning of human and chicken liver HL cDNAs and characterization of a mutation causing human HL deficiency.