Anti-PCCA抗体-抗体-抗体-生物在线
Anti-PCCA抗体

Anti-PCCA抗体

商家询价

产品名称: Anti-PCCA抗体

英文名称: PCCA

产品编号: YB--19909R

产品价格: null

产品产地: 中国/美国

品牌商标: Ybscience

更新时间: 2023-08-17T10:29:50

使用范围: 科研使用

上海钰博生物科技有限公司
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 Anti-PCCA抗体

产品编号 YB-19909R
英文名称 PCCA
中文名称 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/1380元 购买    200ul/2200元 购买    大包装/询价
说 明 书 100ul  200ul
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 Human, Mouse, Rat, 
产品应用 WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 ICC=1:100-500 IF=1:100-500 (石蜡切片需做抗原修复) 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 74kDa
细胞定位 细胞浆 
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human PCCA:451-550/728 
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.
PubMed PubMed
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.

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



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 
 
产品图片
Sample: Kidney (Mouse) Lysate at 40 ug
Primary: Anti-PCCA (bs-19909R) at 1/300 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 74 kD
Observed band size: 74 kD