The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
備考Protein concentration is above or equal to 0.05 mg/ml.
Best use within three months from the date of receipt of this protein.
Concentration information loading...
Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.
pH: 8.00 Constituents: 0.3% Glutathione, 0.79% Tris HCl
3 hydroxy 3 methylglutaryl coenzyme A synthase
3 hydroxy 3 methylglutaryl Coenzyme A synthase 2
3 hydroxy 3 methylglutaryl Coenzyme A synthase 2 (mitochondrial)
3-hydroxy-3-methylglutaryl coenzyme A synthase
HMG CoA synthase
Hydroxymethylglutaryl CoA synthase
Hydroxymethylglutaryl CoA synthase mitochondrial
機能This enzyme condenses acetyl-CoA with acetoacetyl-CoA to form HMG-CoA, which is the substrate for HMG-CoA reductase.
組織特異性High expression in liver and colon. Low expression in testis, heart, skeletal muscle and kidney.
パスウェイMetabolic intermediate biosynthesis; (R)-mevalonate biosynthesis; (R)-mevalonate from acetyl-CoA: step 2/3.
関連疾患Defects in HMGCS2 are the cause of HMG-CoA synthase deficiency (HMGCS deficiency) [MIM:605911]; also known as deficiency of mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase 2. Affected individuals present with severe hypoketotic hypoglycemia, mild hepatomegaly, or fatty liver, and a nondiagnostic pattern of urinary organic acids with increase of medium and short chain dicarboxylic acids.