Anti-HMGCS2 Antibody Picoband®

SKU: A04371-2

Size: 100 μg

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

Form: Lyophilized

Reactivity: Human,Mouse,Rat

Applications: WB,IHC,ICC,IF,Flow Cytometry,ELISA

Application Details: Western blot, 0.1-0.25 μg/ml, Human, Mouse, Rat
Immunohistochemistry(Paraffin-embedded Section), 2-5 μg/ml, Human
Immunocytochemistry/Immunofluorescence, 5 μg/ml, Human
Flow Cytometry (Fixed), 1-3 μg/1x10⁶ cells, Human
ELISA, 0.1-0.5 μg/ml, -

Gene Name: HMGCS2

Specificity:

Background: 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.

Immunogen: E.coli-derived human HMGCS2 recombinant protein (Position: M1-P487).

Clonality: Polyclonal

Contents: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

Purification: Immunogen affinity purified.

Reconstitution: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Reference: 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.

Uniprot ID: P54868

Host: Rabbit

Concentration: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Conjugate:

Cross Reactivity: No cross-reactivity with other proteins.

Isotype: Rabbit IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 50 kDa

Calculated Molecular Weight:

Gene ID: 3158

Protein Name: Hydroxymethylglutaryl-CoA synthase, mitochondrial

Gene Full Name: 3-hydroxy-3-methylglutaryl-CoA synthase 2

Synonyms: HMGCS2; Hydroxymethylglutaryl-CoA synthase; mitochondrial; HMG-CoA synthase; EC 2.3.3.10; 3-hydroxy-3-methylglutaryl coenzyme A synthase