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Key Specifications Table
| Species Reactivity | Key Applications | Host | Format | Antibody Type |
|---|---|---|---|---|
| H | ICC, IP, WB | M | Purified | Monoclonal Antibody |
| Description | |
|---|---|
| Catalogue Number | MAB3480 |
| Brand Family | Chemicon® |
| Trade Name |
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| Description | Anti-CFTR Antibody, a.a. 1370-1380, clone M3A7 |
| Alternate Names |
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| Background Information | CFTR (Cystic Fibrosis Transmembrane Regulator) is an integral membrane protein shown to be involved in the transport of chloride ions. It is found on the surface of the epithelial cells lining the lungs and other organs. CFTR defects are the cause of cystic fibrosis. Cystic Fibrosis (CF) is a common lethal genetic disease caused by mutations of the gene coding for the cystic fibrosis transmembrane conductance factor, a cAMP regulated chloride channel. Approximately 70% of all CF cases share the deletion of a phenylalanine at position 508 (delta F508) which results in abnormal chloride transport. Since the CF mutation is lethal, most often by lung and liver disease, it raises the question of why this genetic disease remains as common as it is. One possible explanation is that Salmonella typhi has been shown to use CFTR to enter intestinal epithelial cells and that delta F508 heterozygote and homozygote mice showed 86% and 100% reductions in S.typhi intestinal submucosal uptake. |
| Product Information | |
|---|---|
| Format | Purified |
| Control |
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| Presentation | Protein A Purified mouse immunoglobulin in 20 mM sodium phosphate, 250 mM NaCl, pH. 7.6, with 0.1% sodium azide as a preservative. |
| Quality Level | MQ100 |
| Applications | |
|---|---|
| Application | Anti-Cystic Fibrosis Transmembrane Conductance Regulator Antibody, a.a. 1370-1380, clone M3A7 detects level of Cystic Fibrosis Transmembrane Conductance Regulator & has been published & validated for use in IC, IP & WB. |
| Key Applications |
|
| Application Notes | Western blot: 1μg/mL, do not boil, recognizes 170kDa CTFR protein. Immunoprecipitation Immunofluorescence T84 adenocarcinoma cells are a good positive control Immunohistochemistry: 1-50μg/mL on acetone fixed frozen sections. Does not work on paraffin embedded tissue. Optimal working dilutions must be determined by the end user. |
| Biological Information | |
|---|---|
| Immunogen | Recombinant protein encoding NBF2 domain of human CFTR |
| Epitope | a.a. 1370-1380 |
| Clone | M3A7 |
| Concentration | Please refer to the Certificate of Analysis for the lot-specific concentration. |
| Host | Mouse |
| Specificity | Recognizes Human cystic fibrosis transmembrane conductance regulator (CFTR). Recognizes an epitope at the C-terminal end of the second nucleotide binding in the region of residues 1370 to 1380. |
| Isotype | IgG1 |
| Species Reactivity |
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| Antibody Type | Monoclonal Antibody |
| Entrez Gene Number |
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| Entrez Gene Summary | 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. |
| Gene Symbol |
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| Non-Reactive Species |
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| Purification Method | Protein A Purfied |
| UniProt Number |
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| UniProt Summary | 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. |
| Molecular Weight | 170 kDa |
| Product Usage Statements | |
|---|---|
| Usage Statement |
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| Storage and Shipping Information | |
|---|---|
| Storage Conditions | Maintain for 1 year at 2–8°C from date of shipment. Aliquot to avoid repeated freezing and thawing. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. |
| Packaging Information | |
|---|---|
| Material Size | 100 µg |