Full Product Name
PCCA Antibody, HRP conjugated
Product Synonym Names
Propionyl-CoA carboxylase alpha chain, mitochondrial; PCCase subunit alpha; Propanoyl-CoA:carbon dioxide ligase subunit alpha; PCCA
Product Gene Name
anti-PCCA antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for P05165
Purity/Purification
>95%, Protein G purified
Immunogen
Recombinant Human Propionyl-CoA carboxylase alpha chain, mitochondrial protein (585-720AA)
Preservative
0.03% Proclin 300
Constituents
50% Glycerol, 0.01M PBS, pH 7.4
Preparation and Storage
Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C or -80 degree C. Avoid repeated freeze.
ISO Certification
Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.
Other Notes
Small volumes of anti-PCCA antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Related Product Information for
anti-PCCA antibody
cytosol, mitochondrial matrix, biotin binding, enzyme binding, propionyl-CoA carboxylase activity, biotin metabolic process, short-chain fatty acid catabolic process
Applications Tested/Suitable for anti-PCCA antibody
ELISA (EIA)
NCBI/Uniprot data below describe general gene information for PCCA. It may not necessarily be applicable to this product.
NCBI Accession #
NP_000273.2
[Other Products]
NCBI GenBank Nucleotide #
NM_000282.3
[Other Products]
UniProt Primary Accession #
P05165
[Other Products]
UniProt Secondary Accession #
Q15979; Q8WXQ7; B4DKY8; B4DPF9; C9JPQ8[Other Products]
UniProt Related Accession #
P05165[Other Products]
Molecular Weight
75,002 Da
NCBI Official Full Name
propionyl-CoA carboxylase alpha chain, mitochondrial isoform a
NCBI Official Synonym Full Names
propionyl-CoA carboxylase alpha subunit
NCBI Official Symbol
PCCA [Similar Products]
NCBI Protein Information
propionyl-CoA carboxylase alpha chain, mitochondrial
UniProt Protein Name
Propionyl-CoA carboxylase alpha chain, mitochondrial
UniProt Synonym Protein Names
Propanoyl-CoA:carbon dioxide ligase subunit alpha
UniProt Gene Name
PCCA [Similar Products]
UniProt Synonym Gene Names
PCCase subunit alpha [Similar Products]
NCBI Summary for PCCA
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.[provided by RefSeq, May 2010]
UniProt Comments for PCCA
PCCA: Defects in PCCA are the cause of propionic acidemia type I (PA-1). PA-1 is a life-threatening disease characterized by episodic vomiting, lethargy and ketosis, neutropenia, periodic thrombocytopenia, hypogammaglobulinemia, developmental retardation, and intolerance to protein. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: Amino Acid Metabolism - valine, leucine and isoleucine degradation; Carbohydrate Metabolism - propanoate; EC 6.4.1.3; Ligase; Mitochondrial
Chromosomal Location of Human Ortholog: 13q32.3
Cellular Component: cytosol; mitochondrial matrix
Molecular Function: biotin binding; enzyme binding
Biological Process: biotin metabolic process; short-chain fatty acid catabolic process
Disease: Propionic Acidemia
Research Articles on PCCA
1. Ten propionic acidemia mutations were confirmed, including 8 affecting the PCCA gene and 2 affecting the PCCB gene
Precautions
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
Disclaimer
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.
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