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PCCA Rabbit anti-Human, Mouse, Polyclonal, Proteintech
SDP

Catalog No. p-7209897 Shop All Proteintech Group Inc Products
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50-173-3670 150 μL
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Catalog No. 50-173-3670 Supplier Proteintech Group Inc Supplier No. 219881AP150UL
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Description

Rabbit Polyclonal Antibody

This antibody is specific to PCCA.

This protein is a key enzyme in the catabolic pathway of odd-chain fatty acids, isoleucine, threonine, methionine, and valine. It is probably a dodecamer composed of six biotin-containing alpha subunits and six beta subunits. The protein is located in the mitochondrial matrix. Defects in PCCA are the cause o fpropionic acidemia type I (PA-1) [MIM:606054]. PA-1 is a life-threatening disease characterized by episodic vomiting, lethargy and ketosis, neutropenia, periodic thrombocytopenia, hypogammaglobulinemia, develop-mental retardation, and intolerance to protein.

Specifications

Antigen PCCA
Applications Immunohistochemistry (Paraffin), Immunoprecipitation, Western Blot
Classification Polyclonal
Concentration 0.26 mg/mL
Conjugate Unconjugated
Formulation PBS with 50% glycerol and 0.02% sodium azide; pH 7.3
Gene PCCA
Gene Accession No. P05165, Q91ZA3
Gene Alias PCCA, PCCase subunit alpha
Gene Symbols PCCA
Host Species Rabbit
Immunogen PCCA Fusion Protein Ag17271
Purification Method Antigen Affinity Chromatography
Quantity 150 μL
Regulatory Status RUO
Primary or Secondary Primary
Gene ID (Entrez) 110821, 5095
Target Species Human, Mouse
Content And Storage -20°C
Product Type Antibody
Form Liquid
Isotype IgG
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