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

The cystic fibrosis transmembrane conductance regulator (CFTR) is a unique member of the ATP-binding cassette (ABC) transporter superfamily that plays a critical role in fluid and electrolyte transport across epithelial tissues. It is composed of two membrane-spanning domain (MSD), nucleotide-binding domain (NBD) motifs linked by a unique regulatory (R) domain. The MSDs assemble to form a transmembrane pore with deep intracellular and shallow extracellular vestibules that funnel anions towards a selectivity filter, which determines the permeation properties of CFTR. The R domain contains multiple consensus phosphorylation sites on the surface of an unstructured domain. Phosphorylation of the R domain stimulates CFTR function by enhancing ATP-dependent channel gating at the NBDs. Thus, it is an anion channel with exquisite regulation. The protein also regulates the function of other channels, such as those that transport positively charged particles called sodium ions across cell membranes. These channels are necessary for the normal function of organs such as the lungs and pancreas. It is expressed on the surface of the epithelial cells that line the lungs and other organs. The pathological role of the protein has been observed in cystic fibrosis and congenital bilateral absence of the vas deferens.\n\nApplications:\nSuitable for use in Immunohistochemistry. Other applications not tested.\n\nRecommended Dilution:\nImmunohistochemistry (Formalin fixed paraffin embedded): 1:50-1:100\nOptimal dilutions to be determined by the researcher.\n\nPositive Control:\nPancreas\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 at least 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

C9040-05B

Size

500ul

Applications

IHC

Hosts

Mouse

Reactivities

Hum

Form

Supplied as a liquid, 0.05% sodium azide.

P Type

Mab

Purity

Ascites

Isotype

IgG1

References

1. Gergely et al. J Biol Chem 29: 21592, 1993. 2. Kartner et al. Nature Genetics 390: 321, 1992. 3. Chen et al. Respir Res Basel, Karger 34: 38-44, 2006.

Additional Info

Recognizes human CFTR.