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货号： bs-19909R-Cy7 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-19909R-Cy7
- 英文名称
- Anti-PCCA/Cy7
- 中文名称
- Cy7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
- Human, Mouse, Rat,
- 产品应用
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: 418774Chicken
Entrez Gene: 738775Chimpanzee
Entrez Gene: 614302Cow
Entrez Gene: 476975Dog
Entrez Gene: 101127469Gorilla
Entrez Gene: 100062251Horse
Entrez Gene: 5095Human
Entrez Gene: 110821Mouse
Entrez Gene: 100456529Orangutan
Entrez Gene: 100524103Pig
Entrez Gene: 687008Rat
Entrez Gene: 699844 Rhesus monkey
Entrez Gene: 437019Zebrafish
Omim: 232000Human
SwissProt: P05165Human
SwissProt: Q91ZA3Mouse
SwissProt: P14882Rat
Unigene: 80741Human
Unigene: 23876Mouse
Unigene: 6033Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.