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Invitrogen™ PCCA Polyclonal Antibody
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Catalog No. PIPA561590
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PIPA561590 100 μL
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Catalog No. PIPA561590 Supplier Invitrogen™ Supplier No. PA561590
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Rabbit Polyclonal Antibody

Immunogen sequence: YPLRHKQADI RINGWAVECR VYAEDPYKSF GLPSIGRLSQ YQEPLHLPGV RVDSGIQPGS DISIYYDPMI SKLITYGSDR TEALKRMADA LDNY Highest antigen sequence identity to the following orthologs: Mouse - 93%, Rat - 84%.

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 propionic acidemia type I (PA-1). 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.
TRUSTED_SUSTAINABILITY

Specifications

Antigen PCCA
Applications Immunohistochemistry (Paraffin), Western Blot
Classification Polyclonal
Concentration 0.1 mg/mL
Conjugate Unconjugated
Formulation PBS with 40% glycerol and 0.02% sodium azide; pH 7.2
Gene PCCA
Gene Accession No. P05165
Gene Alias C79630; Pcca; pccA complementation group; PCCase alpha subunit; PCCase subunit alpha; propanoyl-CoA:carbon dioxide ligase alpha subunit; propanoyl-CoA:carbon dioxide ligase subunit alpha; propionyl CoA carboxylase, alpha polypeptide; propionyl CoA-carboxylase alpha; Propionyl Coenzyme A carboxylase alpha polypeptide; propionyl Coenzyme A carboxylase, alpha polypeptide; propionyl-CoA carboxylase alpha chain, mitochondrial; propionyl-CoA carboxylase alpha subunit; propionyl-Coenzyme A carboxylase, alpha polypeptide; RP11-151A6.1
Gene Symbols PCCA
Host Species Rabbit
Immunogen Recombinant protein corresponding to Human PCCA. Recombinant protein control fragment (Product #RP-97987).
Purification Method Antigen affinity chromatography
Quantity 100 μL
Regulatory Status RUO
Primary or Secondary Primary
Gene ID (Entrez) 5095
Target Species Human
Content And Storage Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
Product Type Antibody
Form Liquid
Isotype IgG
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