Propionyl Coenzyme A Carboxylase Alpha Recombinant Protein | PCCa recombinant protein

Recombinant Propionyl Coenzyme A Carboxylase Alpha (PCCa)

Cat. Num. AAA2034328

• Reactivity: Human
• Applications: SDS-Page, Western Blot, ELISA, Immunoprecipitation
• Purity: >95%
• Synonyms: Propionyl Coenzyme A Carboxylase Alpha; Recombinant Propionyl Coenzyme A Carboxylase Alpha (PCCa); Propanoyl-CoA:carbon dioxide ligase subunit alpha; Propionyl-CoA carboxylase alpha chain; mitochondrial; PCCa recombinant protein

• Host: E Coli
• Reactivity: Human
• Purity/Purification: >95%
• Sequence Length: 728

• Applicable Applications for PCCa recombinant protein: SDS-PAGE, Western Blot (WB), ELISA (EIA), Immunoprecipitation (IP)
• Fragment: Val580~Glu728
• Tag: Prokaryotic expression
• Preparation and Storage: Storage: Avoid repeated freeze/thaw cycles. Store at 2-8 degree C for one month. Aliquot and store at -80 degree C for 12 months.; Stability Test: The thermal stability is described by the loss rate of the target protein. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37 degree C for 48h, and no obvious degradation and precipitation were observed. The loss of this protein is less than 5% within the expiration date under appropriate storage condition.

SDS-Page

NCBI and Uniprot Product Information

• NCBI GI #: 119629439
• NCBI GeneID: 5095
• Molecular Weight: 19.4kDa
• NCBI Official Full Name: propionyl Coenzyme A carboxylase, alpha polypeptide, isoform CRA_c
• NCBI Official Synonym Full Names: propionyl-CoA carboxylase alpha subunit
• NCBI Official Symbol: PCCA
• NCBI Protein Information: propionyl-CoA carboxylase alpha chain, mitochondrial
• UniProt Protein Name: Propionyl-CoA carboxylase alpha chain, mitochondrial
• UniProt Gene Name: PCCA
• UniProt Synonym Gene Names: PCCase subunit alpha
• UniProt Entry Name: PCCA_HUMAN

NCBI Description

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 Description

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: Carbohydrate Metabolism - propanoate; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Ligase; Mitochondrial; EC 6.4.1.3

Chromosomal Location of Human Ortholog: 13q32

Cellular Component: cytosol; mitochondrial matrix

Molecular Function: ATP binding; biotin binding; biotin carboxylase activity; enzyme binding; metal ion binding; propionyl-CoA carboxylase activity

Biological Process: biotin metabolic process; cellular lipid metabolic process; fatty acid beta-oxidation; short-chain fatty acid catabolic process; vitamin metabolic process; water-soluble vitamin metabolic process

Disease: Propionic Acidemia