Product Name	PCCA
Chinese Name	丙酰辅酶A羧化酶α链抗体
Alias	mitochondrial; Pcca; pccA COMPLEMENTATION GROUP; PCCA_HUMAN; PCCase subunit alpha; Propanoyl CoA:carbon dioxide ligase subunit alpha; Propanoyl-CoA:carbon dioxide ligase subunit alpha; Propionyl CoA carboxylase alpha chain; Propionyl CoA carboxylase alpha chain mitochondrial; Propionyl CoA carboxylase alpha polypeptide; Propionyl Coenzyme A Carboxylase Alpha Polypeptide; Propionyl-CoA carboxylase alpha chain.
Research Area	Cell biology  Signal transduction
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	Mouse,  (predicted: Human, Rat, )
Applications	WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （Paraffin sections need antigen repair） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight	74kDa
Cellular localization	cytoplasmic
Form	Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human PCCA: 451-550/728
Lsotype	IgG
Purification	affinity purified by Protein A
Buffer Solution	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Attention	This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed	PubMed
Product Detail	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] Subunit: Probably a dodecamer composed of six biotin-containing alpha subunits and six beta subunits. Subcellular Location: Mitochondrion matrix. DISEASE: Defects in PCCA are the cause of propionic 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, developmental retardation, and intolerance to protein. Similarity: Contains 1 ATP-grasp domain. Contains 1 biotin carboxylation domain. Contains 1 biotinyl-binding domain. SWISS: P05165 Gene ID: 5095 Database links: Entrez Gene: 418774 Chicken Entrez Gene: 738775 Chimpanzee Entrez Gene: 614302 Cow Entrez Gene: 476975 Dog Entrez Gene: 101127469 Gorilla Entrez Gene: 100062251 Horse Entrez Gene: 5095 Human Entrez Gene: 110821 Mouse Entrez Gene: 100456529 Orangutan Entrez Gene: 100524103 Pig Entrez Gene: 687008 Rat Entrez Gene: 699844 Rhesus monkey Entrez Gene: 437019 Zebrafish Omim: 232000 Human SwissProt: P05165 Human SwissProt: Q91ZA3 Mouse SwissProt: P14882 Rat Unigene: 80741 Human Unigene: 23876 Mouse Unigene: 6033 Rat
Product Picture	Sample: Kidney (Mouse) Lysate at 40 ug Primary: Anti-PCCA (SL19909R) at 1/300 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 74 kD Observed band size: 74 kD

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