Ms X CFTR

Species Reactivity	Key Applications	Host	Format	Antibody Type
H	ICC, IP, WB	M	Purified	Monoclonal Antibody

Species Reactivity

Key Applications

Host

Format

Antibody Type

ICC, IP, WB

Purified

Monoclonal Antibody

Description
Catalogue Number	MAB3480
Brand Family	Chemicon®
Trade Name	Chemicon
Description	Anti-CFTR Antibody, a.a. 1370-1380, clone M3A7
Alternate Names	CFTR
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.

Description

Catalogue Number

MAB3480

Brand Family

Chemicon®

Trade Name

Chemicon

Description

Anti-CFTR Antibody, a.a. 1370-1380, clone M3A7

Alternate Names

CFTR

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	T84 whole cell extract
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.

Product Information

Format

Purified

Control

T84 whole cell extract

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.

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	Immunocytochemistry Immunoprecipitation Western Blotting
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.

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

Immunocytochemistry
Immunoprecipitation
Western Blotting

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	Human
Antibody Type	Monoclonal Antibody
Entrez Gene Number	NM_000492.3
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	CFTR ABCC7 MRP7 TNR-CFTR CFTR/MRP CBAVD dJ760C5.1 CF ABC35
Non-Reactive Species	Mouse Rat Shark
Purification Method	Protein A Purfied
UniProt Number	P13569
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

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

Human

Antibody Type

Monoclonal Antibody

Entrez Gene Number

NM_000492.3

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

CFTR
ABCC7
MRP7
TNR-CFTR
CFTR/MRP
CBAVD
dJ760C5.1
CF
ABC35

Non-Reactive Species

Mouse
Rat
Shark

Purification Method

Protein A Purfied

UniProt Number

P13569

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	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.

Product Usage Statements

Usage Statement

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.

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.

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

Packaging Information

Material Size

100 µg

实验耗材

分子生物学试剂

蛋白与免疫学

生化试剂

细胞及检测试剂

色谱及层析

仪器设备

实验服务

办公用品

浏览了该商品的用户最终购买

浏览了该商品的用户还浏览了

特卖

重要规格表

我要咨询