Anti-PCCA Antibody Picoband®

SKU: A03395-1

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,Flow Cytometry,ELISA

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

Gene Name: PCCA

Specificity:

Background: The protein encoded by this gene is the alpha subunit of the heterodimeric mitochondrial enzyme Propionyl-CoA carboxylase. PCCA encodes the biotin-binding region of this enzyme. Mutations in either PCCA or PCCB (encoding the beta subunit) lead to an enzyme deficiency resulting in propionic acidemia. Multiple transcript variants encoding different isoforms have been found for this gene.

Immunogen: E.coli-derived human PCCA recombinant protein (Position: E403-E728). Human PCCA shares 89.6% and 89% amino acid (aa) sequence identity with mouse and rat PCCA, respectively.

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. Bowcock, A. M., Gerken, S. C., Barnes, R. I., Shiang, R., Jabs, E. W., Warren, A. C., Antonarakis, S., Retief, A. E., Vergnaud, G., Leppert, M., Lalouel, J.-M., White, R. L., Cavalli-Sforza, L. L. The CEPH consortium linkage map of human chromosome 13. Genomics 16: 486-496, 1993. 2. Campeau, E., Dupuis, L., Leclerc, D., Gravel, R. A. Detection of a normally rare transcript in propionic acidemia patients with mRNA destabilizing mutations in the PCCA gene. Hum. Molec. Genet. 8: 107-113, 1999. 3. Childs, B., Nyhan, W. L., Borden, M., Bard, L., Cooke, R. E. Idiopathic hyperglycinemia and hyperglycinuria: a new disorder of amino acid metabolism. Pediatrics 27: 522-538, 1961.

Uniprot ID: P05165

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: IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 70 kDa

Calculated Molecular Weight: 52588 MW

Gene ID: 5095

Protein Name: Propionyl-CoA carboxylase alpha chain, mitochondrial

Gene Full Name: propionyl-CoA carboxylase subunit alpha

Synonyms: PCCA; PCCase subunit alpha