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Invitrogen
PCCA Polyclonal Antibody
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Product Details
PA5-98037
Species Reactivity
Host/Isotype
Class
Type
Immunogen
Conjugate
Form
Concentration
Purification
Storage buffer
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Storage conditions
Shipping conditions
RRID
Target Information
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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
References (0)
Bioinformatics
Protein Aliases: 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-CoA carboxylase alpha chain, mitochondrial; RP11-151A6.1
Gene Aliases: C79630; PCCA
UniProt ID: (Human) P05165, (Mouse) Q91ZA3
Entrez Gene ID: (Human) 5095, (Mouse) 110821
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