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Anti-PCCB Antibody Picoband® Fluoro647 Conjugated

Product Specifications

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.

Synonyms

Interleukin-3 receptor subunit alpha; IL-3 receptor subunit alpha; IL-3R subunit alpha; IL-3R-alpha; IL-3RA; CD123; IL3RA; IL3R

Gene Name

PCCB

Gene ID

5096

UniProt

P05166

Host

Rabbit

Reactivity

Human, Mouse, Rat

Cross Reactivity

No cross-reactivity with other proteins.

Immunogen

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

Clonality

Polyclonal

Tissue Specificity

Brain.

Applications

Flow Cytometry

Field of Research

Adaptive Immunity, B Cells, Hematopoietic Progenitors, Immunoglobulins, Immunology, Lymphoid, Receptors, Stem Cells

Purification

Immunogen affinity purified.

Form

Liquid

Function

This is a receptor for interleukin-3.

References & Citations

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.

Storage Conditions

At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.

Applications Notes

6

Gene Name Synonym

Interleukin 3 receptor subunit alpha

Subcellular Location

Membrane. Single-pass type I membrane protein.

Isotype

Rabbit IgG

Contents

Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.

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