Anti-PCCB antibody

Catalog Number:

A001035836STJ

Mfr. No.:

STJ27368

Price:

$297

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Overview
- 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.
  
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Properties
- Host
  
  rabbit
  
  Antibody Type
  
  polyclonal
  
  Isotype
  
  IgG
  
  Reactivity
  
  human; mouse
  
  Immunogen
  
  Recombinant fusion protein containing a sequence corresponding to amino acids 260-539 of human PCCB (NP_000523. 2).
  
  Purification
  
  Affinity purification
  
  Conjugate
  
  Unconjugated
  
  Formulation
  
  PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
  
  Storage
  
  Store at -20°C and avoid freeze-thaw cycles.
  
  Regulatory Status
  
  RUO
  
  Antigen
  
  Propionyl-Coa Carboxylase Beta Chain-Mitochondrial; Pccase Subunit Beta; Propanoyl-Coa-Carbon Dioxide Ligase Subunit Beta (Gene ID:5096; UniProt:P05166)
  
  * For research use only