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HMG-CoA reductase (3-hydroxy-3-methyl-glutaryl-coenzyme A reductase, official symbol HMGCR) is the rate-controlling enzyme (NADH-dependent, EC 1.1.1.88; NADPH-dependent, EC 1.1.1.34) of the mevalonate pathway, the metabolic pathway that produces cholesterol and other isoprenoids.
3-Hydroxy-3-methylglutaryl-CoA lyase deficiency is the result of HMGCL gene mutations. [1] HMGCL is found on chromosome 1p36.11's short arm and codes for the enzyme 3-hydroxymethyl-3-methylglutaryl-coenzyme A lyase (HMG-CoA lyase).
3-Hydroxy-3-methylglutaryl-CoA lyase (or HMG-CoA lyase) is an enzyme (EC 4.1.3.4 that in human is encoded by the HMGCL gene located on chromosome 1. It is a key enzyme in ketogenesis (ketone body formation). It is a ketogenic enzyme in the liver that catalyzes the formation of acetoacetate from HMG-CoA within the mitochondria.
In biochemistry, hydroxymethylglutaryl-CoA synthase or HMG-CoA synthase EC 2.3.3.10 is an enzyme which catalyzes the reaction in which acetyl-CoA condenses with acetoacetyl-CoA to form 3-hydroxy-3-methylglutaryl-CoA (HMG-CoA). This reaction comprises the second step in the mevalonate-dependent isoprenoid biosynthesis pathway.
HMG-CoA synthase: Acetoacetyl-CoA condenses with another Acetyl-CoA molecule to form 3-hydroxy-3-methylglutaryl-CoA (HMG-CoA). HMG-CoA reductase: HMG-CoA is reduced to mevalonate by NADPH. This is the rate limiting step in cholesterol synthesis, which is why this enzyme is a good target for pharmaceuticals . mevalonate-5-kinase
Carnitine Palmitoyl Transferase I & II ( CPT I deficiency & CPT II deficiency) 2,4 Dienoyl-CoA Reductase Deficiency; Electron Transfer Flavoprotein (ETF) Dehydrogenase Deficiency (GA-II/MADD) 3-Hydroxy-3-methylglutaryl-CoA lyase deficiency (HMG deficiency) Very long-chain acyl-coenzyme A dehydrogenase deficiency (VLCAD deficiency)
The systematic name of this enzyme class is (R)-mevalonate:NAD + oxidoreductase (CoA-acylating). [1] Other names in common use include beta-hydroxy-beta-methylglutaryl coenzyme A reductase , beta-hydroxy-beta-methylglutaryl CoA-reductase , 3-hydroxy-3-methylglutaryl coenzyme A reductase , and hydroxymethylglutaryl coenzyme A reductase .
In archaea, HMG-CoA reductase is a cytoplasmic enzyme involved in the biosynthesis of the isoprenoids side chains of lipids. [3] Class I HMG-CoA reductases consist of an N-terminal membrane domain (lacking in archaeal enzymes), and a C-terminal catalytic region. The catalytic region can be subdivided into three domains: an N-domain (N-terminal ...