| 產(chǎn)品編號(hào) | bs-1277R-BF647 |
| 英文名稱 | Rabbit Anti-CFTR/BF647 Conjugated antibody |
| 中文名稱 | BF647標(biāo)記的囊性纖維化跨膜轉(zhuǎn)運(yùn)調(diào)節(jié)因子抗體 |
| 別 名 | ABC 35; ABC35; ABCC 7; ABCC7; ATP binding cassette sub family C member 7; ATP Binding Cassette Superfamily C Member 7; ATP binding cassette transporter sub family C member 7; cAMP dependent chloride channel; CF; CFTR/MRP; Channel conductance controlling ATPase; Cystic fibrosis transmembrane conductance regulator; Cystic fibrosis transmembrane conductance regulator ATP binding cassette sub family C member 7; ATP-binding cassette sub-family C member 7; cAMP-dependent chloride channel; CFTR; CFTR_HUMAN; Channel conductance-controlling ATPase; Cystic Fibrosis Transmembrane Regulator; Cystic Fibrosis Transmembrane Regulator; dJ760C5.1; MRP 7; MRP7; TNR CFTR. |
| 規(guī)格價(jià)格 | 100ul/2980元 購買 大包裝/詢價(jià) |
| 說 明 書 | 100ul |
| 研究領(lǐng)域 | 細(xì)胞生物 免疫學(xué) 新陳代謝 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | (predicted: Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep, ) |
| 產(chǎn)品應(yīng)用 | IF=1:50-200
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 168kDa |
| 性 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human CFTR |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲(chǔ) 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存條件 | 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. |
| 產(chǎn)品介紹 |
background: This gene encodes a member of the ATP-binding cassette (ABC) transporter superfamily. ABC proteins transport various molecules across extra- and intra-cellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White). This protein is a member of the MRP subfamily that is involved in multi-drug resistance. The encoded protein functions as a chloride channel and controls the regulation of other transport pathways. Mutations in this gene are associated with the autosomal recessive disorders cystic fibrosis and congenital bilateral aplasia of the vas deferens. Alternatively spliced transcript variants have been described, many of which result from mutations in this gene. [provided by RefSeq, Jul 2008] Function: Involved in the transport of chloride ions. May regulate bicarbonate secretion and salvage in epithelial cells by regulating the SLC4A7 transporter. Can inhibit the chloride channel activity of ANO1. Subunit: Interacts with SHANK2 (By similarity). Interacts with SLC9A3R1, MYO6 and GOPC. Interacts with SLC4A7 through SLC9A3R1. Found in a complex with MYO5B and RAB11A. Interacts with ANO1. Subcellular Location: Early endosome membrane; Multi-pass membrane protein. Cell membrane. Tissue Specificity: Found on the surface of the epithelial cells that line the lungs and other organs. Post-translational modifications: Phosphorylated; activates the channel. It is not clear whether PKC phosphorylation itself activates the channel or permits activation by phosphorylation at PKA sites. Phosphorylated by AMPK. Ubiquitinated, leading to its degradation in the lysosome. Deubiquitination by USP10 in early endosomes, enhances its endocytic recycling. DISEASE: Cystic fibrosis (CF) [MIM:219700]: A common generalized disorder of the exocrine glands which impairs clearance of secretions in a variety of organs. It is characterized by the triad of chronic bronchopulmonary disease (with recurrent respiratory infections), pancreatic insufficiency (which leads to malabsorption and growth retardation) and elevated sweat electrolytes. It is the most common genetic disease in Caucasians, with a prevalence of about 1 in 2'000 live births. Inheritance is autosomal recessive. Note=The disease is caused by mutations affecting the gene represented in this entry. Congenital bilateral absence of the vas deferens (CBAVD) [MIM:277180]: Important cause of sterility in men and could represent an incomplete form of cystic fibrosis, as the majority of men suffering from cystic fibrosis lack the vas deferens. Note=The disease is caused by mutations affecting the gene represented in this entry. Similarity: Belongs to the ABC transporter superfamily. ABCC family. CFTR transporter (TC 3.A.1.202) subfamily. Contains 2 ABC transmembrane type-1 domains. Contains 2 ABC transporter domains. Database links: Entrez Gene: 1080 Human Entrez Gene: 12638 Mouse Omim: 602421 Human SwissProt: P13569 Human SwissProt: P26361 Mouse Unigene: 489786 Human Unigene: 621460 Human Unigene: 661104 Human Unigene: 15621 Mouse Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. CFTR是一種獨(dú)特的氯離子通道，盡管稱為氯離子通道，但在結(jié)構(gòu)上卻明顯地不同于其他氯離子通道，屬于一種ATP結(jié)合盒轉(zhuǎn)運(yùn)體(ATP-binding cassettetransporter, ABC)家族。CFTR主要為氯離子跨上皮運(yùn)動(dòng)提供了選擇性通道，對(duì)于跨上皮的鹽類運(yùn)輸、液體流動(dòng)和離子濃度調(diào)節(jié)等都具有重要的決定作用。 CFTR分布廣泛，許多器官，如肺、肝、胰腺、腸、生殖腺等的細(xì)胞膜中都有表達(dá)，盡管稱為氯離子通道，但還涉及到其他一價(jià)陰離子的運(yùn)輸，由于生理?xiàng)l件下氯離子最為重要，故稱為氯離子通道，近年來，它一直是離子通道研究中的一個(gè)熱點(diǎn)。 |
