Rabbit anti-Human PCCA Polyclonal Antibody | anti-PCCA antibody

PCCA, ID (PCCA, Propionyl-CoA carboxylase alpha chain, mitochondrial, Propanoyl-CoA:carbon dioxide ligase subunit alpha)

Cat. Num. AAA6005102

• Reactivity: Human
• Applications: ELISA, Western Blot, Immunohistochemistry
• Purity: Affinity Purified; Purified by Protein A affinity chromatography.
• Synonyms: PCCA; Polyclonal Antibody; ID (PCCA; Propionyl-CoA carboxylase alpha chain; mitochondrial; Propanoyl-CoA:carbon dioxide ligase subunit alpha); Anti -PCCA; anti-PCCA antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Human
• Purity/Purification: Affinity Purified; Purified by Protein A affinity chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2, 0.09% sodium azide.

• Applicable Applications for anti-PCCA antibody: ELISA (EL/EIA), Western Blot (WB), Immunohistochemistry (IHC)
• Application Notes: Suitable for use in Western Blot, Immunohistochemistry, ELISA; Dilution: ELISA: 1:1,000; Western Blot: 1:100-500; Immunohistochemistry: 1:10-50
• Immunogen: PCCA antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 336-365 amino acids from the Central region of human PCCA.
• Preparation and Storage: May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(PCCA Antibody (Center) western blot analysis in A549 cell line lysates (35ug/lane).This demonstrates the PCCA antibody detected the PCCA protein (arrow).)

Immunohistochemistry (IHC)

(PCCA Antibody (Center) immunohistochemistry analysis in formalin fixed and paraffin embedded human breast tissue followed by peroxidase conjugation of the secondary antibody and DAB staining.This data demonstrates the use of PCCA Antibody (Center) for immunohistochemistry. Clinical relevance has not been evaluated.)

Related Product Information for anti-PCCA antibody

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.

Product Categories/Family for anti-PCCA antibody

Antibodies; Abs to Mitochondrial Proteins

NCBI and Uniprot Product Information

• NCBI GI #: 295821183
• NCBI GeneID: 5095
• NCBI Accession #: NP_001171475.1
• NCBI GenBank Nucleotide #: NM_001178004.1
• UniProt Accession #: P05165; Q15979; Q8WXQ7; B4DKY8; B4DPF9; C9JPQ8
• Molecular Weight: 80,059 Da
• NCBI Official Full Name: propionyl-CoA carboxylase alpha chain, mitochondrial isoform c
• NCBI Official Synonym Full Names: propionyl CoA carboxylase, alpha polypeptide
• NCBI Official Symbol: PCCA
• NCBI Protein Information: propionyl-CoA carboxylase alpha chain, mitochondrial; PCCase alpha subunit; pccA complementation group; propanoyl-CoA:carbon dioxide ligase alpha subunit; propionyl Coenzyme A carboxylase, alpha polypeptide
• 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: EC 6.4.1.3; Mitochondrial; Carbohydrate Metabolism - propanoate; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Ligase

Chromosomal Location of Human Ortholog: 13q32

Cellular Component: mitochondrial matrix; cytosol

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

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

Disease: Propionic Acidemia