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重要规格表
品种反应性 主要应用 宿主 格式 抗体类型 H WB, ICC M Purified Monoclonal Antibody 描述 产品目录编号 05-581 品牌系列 Upstate
商名 - Upstate
描述 Anti-CFTR Antibody, clone MM13-4 Alternate Names - cystic fibrosis transmembrane conductance regulator, ATP-binding cassette (sub-family C, member 7)
- ATP-binding cassette sub-family C, member 7
- ATP-binding cassette transporter sub-family C member 7
- cAMP-dependent chloride channel
- cystic fibrosis transmembrane conductance regulator
- cystic fibrosis transmembrane conductance regulator (ATP-binding cassette sub-family C, member 7)
- cystic fibrosis transmembrane conductance regulator (ATP-binding cassette sub-family C
- member 7)
背景信息 CFTR (Cystic Fibrosis Transmembrane conductance Regulator) is a 170 kDa chloride (Cl-) channel that regulates salt and water transport across epithelial tissues, where it modulates production of mucus, sweat, saliva, tears, and digestive enzymes. Cystic fibrosis patients have mutations in this protein, resulting in defective in ion transport. Symptoms include reduced transport of sodium chloride (saltier sweat) and digestive and respiratory disorders caused by thicker mucus. CFTR mutations are also associated with congenital bilateral absence of the vas deferens in males. 产品信息 格式 Purified 控制 - Pancreas. RIPA cell lysates
演示 Purified mouse monoclonal IgG1k in buffer containing 0.02M phosphate buffer, pH 7.6, 0.25M NaCl, and 0.1% sodium azide. 应用 应用 Anti-CFTR Antibody, clone MM13-4 detects level of CFTR & has been published & validated for use in IC, IP & WB. 主要应用 - Western Blotting
- Immunocytochemistry
应用说明 Immunoprecipitation:
This antibody has been shown to immunoprecipitate CFTR from a T84 cell lysate by an independent laboratory.
Immunocytochemistry:
This antibody has been reported to immunostain CFTR in T84 cells.生物信息 免疫原品种 Synthetic peptide (RKGYRQRLELSD) corresponding to residues 25-36 of human cystic fibrosis transmembrane conductance regulator (CFTR). The immunizing sequence is identical in monkey and rabbit, has 11/12 identical amino acids in sheep, cow and frog, 10/12 identity in mouse and 9/12 identity in rat. Clone MM13-4. 克隆 MM13-4 浓缩 Please refer to the Certificate of Analysis for the lot-specific concentration. 宿主 Mouse 特异性 This antibody recognizes CFTR, Mr 170kDa. Two additional proteins were detected, Mr 52 and 38 kDa, in CFTR-transfected BHK cell lysates. 同种型 IgG1κ 品种反应性 Human Species Reactivity Note Human, other species cross-reactivity not tested. 抗体类型 Monoclonal Antibody Entrez基因编号 - NM_000492
Entrez基因汇总 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. 基因符号 - TNR-CFTR
- CFTR
- CFTR/MRP
- ABCC7
- CBAVD
- dJ760C5.1
- CF
- MRP7
- ABC35
纯化方法 Protein A purfied UniProt编号 - P13569
UniProt汇总 FUNCTION: SwissProt: P13569 # Involved in the transport of chloride ions. May regulate bicarbonate secretion and salvage in epithelial cells by regulating the SLC4A7 transporter.
SIZE: 1480 amino acids; 168142 Da
SUBUNIT: Interacts with SHANK2 (By similarity). Interacts with SLC9A3R1, MYO6 and GOPC. Interacts with SLC4A7 through SLC9A3R1.
SUBCELLULAR LOCATION: Membrane; Multi-pass membrane protein.
TISSUE SPECIFICITY: Found on the surface of the epithelial cells that line the lungs and other organs.
DOMAIN: SwissProt: P13569 The PDZ-binding motif mediates interactions with GOPC and with the SLC4A7, SLC9A3R1/EBP50 complex.
DISEASE: SwissProt: P13569 # Defects in CFTR are the cause of cystic fibrosis (CF) [MIM:219700]; also known as mucoviscidosis. CF is the most common genetic disease in the Caucasian population, with a prevalence of about 1 in 2000 live births. Inheritance is autosomal recessive. CF is a common generalized disorder of exocrine gland function 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. & Defects in CFTR are the cause of congenital bilateral absence of the vas deferens (CBAVD) [MIM:277180]. CBAVD is an 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.
SIMILARITY: Belongs to the ABC transporter family. CFTR transporter (TC 3.A.1.202) subfamily. & Contains 2 ABC transmembrane type-1 domains. & Contains 2 ABC transporter domains.产品使用声明 质量保证 Routinely evaluated by western blot on human T84 colon carcinoma epithelial RIPA cell lysates or CFTR-transfected BHK.
Western Blot Analysis:
0.5-2 μg/mL of this lot detected CFTR in human T84 colon carcinoma epithelial RIPA cell lysates. A previous lot detected CFTR in CFTR-transfected BHK.
Note: CFTR can run as a diffuse protein on SDS-PAGE.使用声明 - Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
储存和货运信息 存储条件 Stable for 1 year at 2-8°C from date of receipt. 包装信息 数量 200 µg
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