Cystic Fibrosis Transmembrane Conductance Regulator (ATP-Binding Cassette Sub-Family C, Member 7) (CFTR) antibody

Antigen: Cystic Fibrosis Transmembrane Conductance Regulator (ATP-Binding Cassette Sub-Family C, Member 7) (CFTR)

Synonyms: CF, MRP7, ABC35, ABCC7, CFTR/MRP, TNR-CFTR, dJ760C5.1, CFTR

Clonality: Monoclonal (M3A7)

Host: Mouse

Reactivity: Human

Application: Immunohistochemistry (Frozen Sections) (IHC (fro)), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))

Catalog no.: ABIN115198

Additional Information

Characteristics: Synonyms: Cystic fibrosis transmembrane conductance regulator, Channel conductance-controllingATPase, cAMP-dependent chloride channel, ATP-binding cassette transporter sub-family Cmember 7

Alternative name: ABCC7 / CFTR

Swiss-Prot: P13569

Immunogen: BALB/C mice were immunized with recombinant protein encoding NBF2 domain of humanCFTR.

Isotype: IgG1  (Matching secondary antibodies)

Clone: M3A7

Description: Cystic Fibrosis Transmembrane Regulator (CFTR) is composed of two membrane-spanningdomains (MSD), two nucleotide-binding domains (NBD) and one R domain. It is structurallysimilar to multidrug resistance protein (Mdr1). CFTR is found in the apical membranes ofepithelia within the pancreas, intestine, bile duct, sweat gland and male genital ducts. CFTR is a valuable marker of human pancreatic duct cell development and differentiation. CFTR mutations can also result in congenital bilateral absence of vas deferens (CBAVD), aform of male sterility that a majority of male CF patients exhibit. Since the CF mutation is lethal, most often by lung and liver disease, it raises the questionof why this genetic disease remains as common as it is. One possible explanation is thatSalmonella typhi has been shown to use CFTR to enter intestinal epithelial cells and thatdelta F508 heterozygote and homozygote mice showed 86% and 100% reductions in S. typhi intestinal submucosal uptake.

Specificity: This antibody detects ABCC7/CFTRIt reacts with a protein of 165-170 kD. Cellular Localization: Cell membraneSpecies: Human. Others not tested.

Application Details

Application Notes: Immunohistochemistry on Paraffin Embedded Sections: Use a dilution of 1/50-1/100 in anABC method (30 minutes at room temperature). Formalin fixed paraffin embedded tissue sections require high temperature antigenunmasking with 10 mM citrate buffer, pH 6.0 prior to immunostaining. Recommended positive control: Pancreas. Other applications not tested. Optimal dilutions are dependent on conditions and should be determined by the user.

Storage: Store the antibody undiluted at 2-8°C for one month or (in aliquots) at -20°C for longer. Avoid repeated freezing and thawing. Shelf life: one year from despatch.

Restrictions: For Research Use only

Images

Formalin fixed paraffin embedded human pancreas stained with CFTR antibody ABIN115197

Publications

Publications: Kartner, Augustinas, Jensen et al.: "Mislocalization of delta F508 CFTR in cystic fibrosis sweat gland." in: Nature genetics, Vol. 1, Issue 5, pp. 321-7, 1993 (PubMed).