| 产品编号 | bs-19909R-BF647 |
| 英文名称 | Anti-PCCA/BF647 |
| 中文名称 | BF647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 | ICC=1:50-200 IF=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: 101127469 Gorilla Entrez Gene: 100062251 Horse Entrez Gene: 5095 Human Entrez Gene: 110821 Mouse Entrez Gene: 100456529 Orangutan Entrez Gene: 699844 Rhesus monkey Entrez Gene: 437019 Zebrafish Omim: 232000 Human SwissProt: P05165 Human SwissProt: Q91ZA3 Mouse 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. |
| 1、抗体溶解方法 | |
| 2、抗体修复方式 | |
| 3、常用试剂的配制 | |
| 4、免疫组化操作步骤 | |
| 5、免疫组化问题解答 | |
| 6、Western Blotting 操作步骤 | |
| 7、Western Blotting 问题解答 | |
| 8、关于肽链的设计 | |
| 9、多肽的溶解与保存 | |
| 10、酶标抗体效价测定程序 | |
