Product Name	PCCB
Chinese Name	丙酰辅酶A羧化酶β链抗体
Alias	DKFZp451E113; PCCase subunit beta; pccB; PCCB_HUMAN; pccBC Complementation group; Propanoyl CoA:carbon dioxide ligase subunit beta; Propanoyl-CoA:carbon dioxide ligase subunit beta; Propionyl CoA carboxylase beta chain, mitochondrial; propionyl Coenzyme A carboxylase, beta polypeptide; Propionyl-CoA carboxylase , beta subunit; Propionyl-CoA carboxylase beta chain, mitochondrial; R74805.
Research Area	Tumour  Cell biology  Signal transduction
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	Mouse,  (predicted: Human, Rat, Chicken, Dog, Pig, Cow, Horse, Sheep, )
Applications	WB=1:500-2000 ELISA=1:5000-10000  not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight	58kDa
Cellular localization	cytoplasmic
Form	Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human PCCB: 321-420/539
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 a subunit of the propionyl-CoA carboxylase (PCC) enzyme, which is involved in the catabolism of propionyl-CoA. PCC is a mitochondrial enzyme that probably acts as a dodecamer of six alpha subunits and six beta subunits. This gene encodes the beta subunit of PCC. Defects in this gene are a cause of propionic acidemia type II (PA-2). 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 PCCB are the cause of propionic acidemia type II (PA-2) [MIM:606054]. PA-2 is a life-threatening disease characterized by episodic vomiting, lethargy and ketosis, neutropenia, periodic thrombocytopenia, hypogammaglobulinemia, developmental retardation, and intolerance to protein. Similarity: Belongs to the AccD/PCCB family. Contains 1 carboxyltransferase domain. SWISS: P05166 Gene ID: 5096 Database links: Entrez Gene: 5096 Human Entrez Gene: 66904 Mouse Entrez Gene: 24624 Rat Omim: 232050 Human SwissProt: Q2TBR0 Cow SwissProt: P05166 Human SwissProt: Q99MN9 Mouse SwissProt: P79384 Pig SwissProt: P07633 Rat Unigene: 63788 Human Unigene: 335385 Mouse Unigene: 9732 Rat
Product Picture	Sample: Liver (Mouse) Lysate at 40 ug Primary: Anti-PCCB (SL19910R)at 1/300 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 58kD Observed band size: 58kD

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