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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 | MAB3484 |
| Brand Family | Chemicon® |
| Trade Name |
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| Description | Anti-Cystic Fibrosis Transmembrane Conductance Regulator Antibody, a.a. 386-412, clone L12B4 |
| Alternate Names |
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| Product Information | |
|---|---|
| Format | Purified |
| Presentation | Purified immunoglobulin. Liquid in 0.02 M Phosphate buffer, 0.25 M NaCl, pH 7.6 with 0.1% sodium azide. |
| Quality Level | MQ100 |
| Applications | |
|---|---|
| Application | Anti-Cystic Fibrosis Transmembrane Conductance Regulator Antibody, a.a. 386-412, clone L12B4 is an antibody against Cystic Fibrosis Transmembrane Conductance Regulator for use in IC, IP & WB. |
| Key Applications |
|
| Application Notes | Western blot: 1-10μg/mL, note do not boil the lysate, incubate at 80C for 30 minutes prior to running SDS-PAGE. CTFR aggregates upon boiling. Antibody recognizes CFTR at 170kDa and two additional proteins at 100 & 140kDa. Immunoprecipitation Immunofluorescence Note: Does not work on paraffin embedded tissue. Optimal working dilutions must be determined by the end user. |
| Biological Information | |
|---|---|
| Epitope | a.a. 386-412 of human CFTR |
| Clone | L12B4 |
| Concentration | Please refer to the Certificate of Analysis for the lot-specific concentration. |
| Host | Mouse |
| Specificity | Human cystic fibrosis transmembrane conductance regulator (CFTR). Recognizes an epitope at the cytoplasmic region preceding the first nucleotide binding domain between residues 386 to 412. Recognizes CFTR, Mr 170kDa and two additional proteins at ~ Mr 100 and 140kDa. |
| Isotype | IgG2a |
| 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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| 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. |
| Product Usage Statements | |
|---|---|
| Usage Statement |
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| Storage and Shipping Information | |
|---|---|
| Storage Conditions | Maintain at 2-8°C in undiluted aliquots up to 6 months. |
| Packaging Information | |
|---|---|
| Material Size | 100 µg |