3-Hydroxy-3-methylglutaryl-CoA lyase deficiency, (HMGCLD) also known as HMGCL deficiency, HMG-CoA lyase deficiency, or hydroxymethylglutaric aciduria,...

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...

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...

variety of end-products, including cholesterol in humans. HMG-CoA lyase breaks it into acetyl CoA and acetoacetate. Steroidogenic enzyme Sarkar DP (2015)....

congestion Hepatic failure Hereditary ACTH resistance HMG-CoA lyase deficiency Hydroxymethylglutaryl-CoA lyase deficiency Hyperinsulinism-hyperammonemia syndrome...

isocitrate lyase EC 4.1.3.2: Now EC 2.3.3.9, malate synthase EC 4.1.3.3: N-acetylneuraminate lyase EC 4.1.3.4: hydroxymethylglutaryl-CoA lyase EC 4.1.3...

or if they are not tolerated. Statins competitively inhibit hydroxymethylglutaryl (HMG) CoA reductase which is used in the biosynthesis of cholesterol...

or hydroxymethylglutaryl-CoA (HMG-CoA) respectively. MC-CoA is then converted by the enzyme methylcrotonyl-CoA carboxylase to methylglutaconyl-CoA (MG-CoA)...

or hydroxymethylglutaryl-CoA (HMG-CoA) respectively. MC-CoA is then converted by the enzyme methylcrotonyl-CoA carboxylase to methylglutaconyl-CoA (MG-CoA)...

> 1 in 75,000 Hydroxymethylglutaryl lyase deficiency (HMG) < 1 in 100,000 Isovaleric acidemia (IVA) < 1 in 100,000 3-Methylcrotonyl-CoA carboxylase deficiency...