Cystic Fibrosis Transmembrane Conductance Regulator (CFTR, CF, CFTR/MRP, ABC35, ATP-binding Cassette Sub-family C Member 7, ATP Binding Cassette Superfamily C Member 7, ABCC7, cAMP-dependent Chloride Channel, Channel Conductance-controlling ATPase, dJ760C

CFTR (cystic fibrosis transmembrane conductance regulator) is composed of two membrane-spanning domains (MSD), two nucleotide-binding domains (NBD), and an R domain. It is structurally similar to multidrug resistance (Mdr1) protein and both are members of the superfamily of ATP-binding cassette (ABC) transporters, also known as traffic ATPases, which are implicated in the movement of various substrates. The CFTR protein is a small conductance adenosine 3',5'-cyclic monophosphate (cAMP)-activated chloride ion channel found in the apical membranes of epithelia within the pancreas, airway, intestine, bile duct, sweat gland, and male genital ducts. CFTR is a valuable marker of human pancreatic duct cell development and differentiation.\n\nApplications:\nSuitable for use in Immunohistochemistry, Immunoprecipitation and Western Blot. Other applications not tested. \n\nRecommended Dilutions:\nImmunohistochemistry (Frozen): The epitope is sensitive to formaldehyde fixation and tissue processing. Acetone fixation is recommended for frozen sections. Not recommeneded for use in paraffin sections.\nWestern Blot: 10ug/ml detects a band at 170kD under native conditions. Do not boil the lysate. Instead incubate at 37 degrees C for 30 minutes. CFTR can run as a diffuse protein on SDS-PAGE.\nOptimal dilutions to be determined by the researcher. \n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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SPECIFICATIONS

Catalog Number

C9040-05D

Size

100ug\n

Applications

IHC, IP, WB

Hosts

Mouse

Reactivities

Hum

Form

Supplied as a liquid in PBS, 0.1% sodium azide.

P Type

Mab

Purity

Purified

Isotype

IgG1

References

1. Kartner, N. et al. (1992) Mislocalization of delta F508 CFTR in cystic fibrosis sweat gland. Nat Genet. 1: 321-327. 2. Kartner, N. and Riordan, J.R. (1998) Characterization of polyclonal and monoclonal antibodies to cystic fibrosis transmembrane conductance regulator. Methods Enzymol. 292: 629-652 3. Chang, X.B. et al (1993) Protein kinase A (PKA) still activates CFTR chloride channel after mutagenesis of all 10 PKA consensus phosphorylation sites. J Biol Chem. 268: 11304-11311.4. Zhang, F. et al. (1998) Limited proteolysis as a probe for arrested conformational maturation of delta F508 CFTR. Nat Struct Biol. 5: 180-183.

Additional Info

Recognizes human CFTR. Recognizes an epitope in the ABC transporter 2 domain, specifically to a region between aa1370-1380. Does not react with mouse

Alternative Names

ATP Binding Cassette Transporter Sub-family C Member 7, Cystic Fibrosis Transmembrane Regulator