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Rabbit Anti-PCCA/AP Conjugated antibody (bs-19909R-AP)
~~~促销,代码KXJ230206-6~~~
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-19909R-AP
英文名称 Anti-PCCA/AP
中文名称 碱性磷酸酶(AP)标记的丙酰辅酶A羧化酶α链抗体
别    名 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.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 细胞生物  信号转导  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 (predicted: Human, Mouse, Rat, )
产品应用 WB=1:50-200 IHC-P=1:50-200 IHC-F=1:50-200 ICC=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 74kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human PCCA
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
产品介绍 background:
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.

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



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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