Anti-HMGCS2 Antibody Picoband®

3-hydroxy-3-methylglutaryl-CoA synthase 2 (mitochondrial) is an enzyme in humans that is encoded by the HMGCS2 gene. The protein encoded by this gene belongs to the HMG-CoA synthase family. It is a mitochondrial enzyme that catalyzes the first reaction of ketogenesis, a metabolic pathway that provides lipid-derived energy for various organs during times of carbohydrate deprivation, such as fasting. Mutations in this gene are associated with HMG-CoA synthase deficiency. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.

Urokinase-type plasminogen activator; U-plasminogen activator; uPA; Urokinase-type plasminogen activator long chain A; Urokinase-type plasminogen activator short chain A; Urokinase-type plasminogen activator chain B; PLAU

1. Aledo, R., Zschocke, J., Pie, J., Mir, C., Fiesel, S., Mayatepek, E., Hoffmann, G. F., Casals, N., Hegardt, F. G. Genetic basis of mitochondrial HMG-CoA synthase deficiency. Hum. Genet. 109: 19-23, 2001. 2. Ayte, J., Gil-Gomez, G., Haro, D., Marrero, P. F., Hegardt, F. G. Rat mitochondrial and cytosolic 3-hydroxy-3-methylglutaryl-CoA synthases are encoded by two different genes. Proc. Nat. Acad. Sci. 87: 3874-3878, 1990. 3. Bouchard, L., Robert, M.-F., Vinarov, D., Stanley, C. A., Thompson, G. N., Morris, A., Leonard, J. V., Quant, P., Hsu, B. Y. L., Boneh, A., Boukaftane, Y., Ashmarina, L., Wang, S., Miziorko, H., Mitchell, G. A. Mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase deficiency: clinical course and description of causal mutations in two patients. Pediat. Res. 49: 326-331, 2001.

At -20°C for one year from date of receipt. After reconstitution, at 4°C for one month. It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freezing and thawing.