To order chemicals, medical devices, or other restricted products please provide ID that includes your business name & shipping address via email [email protected] or fax 484.881.5997 referencing your VWR account number. Acceptable forms of ID are:
- • State issued document with your organization's Federal Tax ID Number
- • State issued document with your organization's Resale Tax ID Number
- • City or County issued Business License
- • State Department of Health Services License
- • Any other ID issued by the State that includes the business name & address
* ATTN: California Customers may require additional documentation as part of the CA Health & Safety Code. Products that fall under this regulation will be placed on a mandatory 21-day hold after documentation is received. VWR will not lift restrictions for residential shipping addresses.
Specifications
- Antibody type:Primary
- Antigen name:Propionyl CoA Carboxylase Alpha
- Clonality:Polyclonal
- Host:Rabbit
- Isotype:IgG
- Reactivity:Human,Mouse
- Antigen symbol:PCCA
- Conjugation:Unconjugated
- ELISA:Yes
- ImmunoChemistry:Yes
- Size:150 µL
- Western blot:Yes
- Antigen synonyms:RP11-151A6.1
- Storage buffer:PBS with 0.02% sodium azide and 50% glycerol pH 7.3.
- Shipping temperature:Store at -20°C
- Immunogen:Fusion Protein
- Purification:Antigen affinity Purification
- Supplier No.:21988-1-AP
Specifications
About this item
Propionyl-CoA carboxylase (PCC) catalyzes the biotin-dependent carboxylation of propionyl-CoA to D-methyl-malonyl CoA, a reaction that occurs in the mitochondrial matrix. PCC is involved in the catabolism of several essential amino acids (methionine, isoleucine, threonine and valine), as well as odd chain fatty acids and cholesterol. Deficiency of PCC results in propionic acidemia, a metabolic disorder characterized by severe metabolic ketoacidosis, vomiting, lethargy and hypotonia. PCC consists of nonidentical subunits (α and β) encoded by different genes (PCCA and PCCB, respectively). The αPCC cDNA contains an open reading frame of 2106 nucleotide bases and codes for a 702 amino acid polypeptide. The mature length subunit is 70 kDa and contains the biotin binding site.. This protein has 3 isoforms produced by alternative splicing with the molecular weight of 80 kDa, 77 kDa and 75 kDa. The full length protein has a transit peptide with 52 amino acids. This antibody is specific to PCCA.
Western Blot:L02 Cells, 1:500-1:5000; IHC: Human Liver, 1:20-1:200
Type: Primary
Antigen: PCCA
Clonality: Polyclonal
Clone:
Conjugation: Unconjugated
Epitope:
Host: Rabbit
Isotype: IgG
Reactivity: Human, Mouse