Anti-PCCB Antibody Picoband®

SKU: A04326-1

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,IF,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
Immunofluorescence, 5 μg/ml, Human
Flow Cytometry (Fixed), 1-3 μg/1x10⁶ cells, Human
ELISA, 0.1-0.5 μg/ml, -

Gene Name: PCCB

Specificity:

Background: The protein encoded by this gene is a subunit of the propionyl-CoA carboxylase (PCC) enzyme, which is involved in the catabolism of propionyl-CoA. PCC is a mitochondrial enzyme that probably acts as a dodecamer of six alpha subunits and six beta subunits. This gene encodes the beta subunit of PCC. Defects in this gene are a cause of propionic acidemia type II (PA-2). Multiple transcript variants encoding different isoforms have been found for this gene.

Immunogen: E.coli-derived human PCCB recombinant protein (Position: D83-L539).

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. Chloupkova, M., Maclean, K. N., Alkhateeb, A., Kraus, J. P. Propionic acidemia: analysis of mutant propionyl-CoA carboxylase enzymes expressed in Escherichia coli. Hum. Mutat. 19: 629-640, 2002. 2. Desviat, L. R., Clavero, S., Perez-Cerda, C., Navarrete, R., Ugarte, M., Perez, B. New splicing mutations in propionic acidemia. J. Hum. Genet. 51: 992-997, 2006. 3. Fenton, W. A., Gravel, R. A., Rosenblatt, D. S. Disorders of propionate and methylmalonate metabolism.In: Scriver, C. R.; Beaudet, A. L.; Sly, W. S.; Valle, D. (eds.) : The Metabolic and Molecular Bases of Inherited Disease. Vol. II. (8th ed.) New York: McGraw-Hill (pub.) 2001. P. 2176.

Uniprot ID: P05166

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: 55 kDa

Calculated Molecular Weight:

Gene ID: 5096

Protein Name: Propionyl-CoA carboxylase beta chain, mitochondrial

Gene Full Name: propionyl-CoA carboxylase subunit beta

Synonyms: PCCase subunit beta; PCCB