Anti-CFTR Antibody

The cystic fibrosis transmembrane conductance regulator (CFTR) protein is a member of the ATP-binding cassette (ABC) transporter superfamily, and a member of the MRP subfamily that is involved in multi-drug resistance (1,2) . CFTR functions as a chloride channel and controls the regulation of other transport pathways (3) . Mutations in this gene are associated with the autosomal recessive disorder cystic fibrosis, the most common, fatal, inherited disease of caucasian populations (1) .

CF, MRP7, ABC35, ABCC7, CFTR/MRP, TNR-CFTR, dJ760C5.1, Cystic fibrosis transmembrane conductance regulator, ATP-binding cassette sub-family C member 7, CFTR

CFTR antibody was raised against an 18 amino acid peptide near the carboxy terminus of human CFTR. The immunogen is located within amino acids 1290 - 1340 of CFTR.

Expressed in the respiratory airway, including bronchial epithelium, and in the female reproductive tract, including oviduct (at protein level) (PubMed:22207244, PubMed:15716351) . Detected in pancreatic intercalated ducts in the exocrine tissue, on epithelial cells in intralobular striated ducts in sublingual salivary glands, on apical membranes of crypt cells throughout the small and large intestine, and on the reabsorptive duct in eccrine sweat glands (PubMed:1284548, PubMed:28130590) . Detected on the equatorial segment of the sperm head (at protein level) (PubMed:19923167) . Detected in nasal and bronchial superficial epithelium (PubMed:15716351) . Expressed by the central cells on the sebaceous glands, dermal adipocytes and, at lower levels, by epithelial cells (PubMed:28130590) .

Epithelial ion channel that plays an important role in the regulation of epithelial ion and water transport and fluid homeostasis (PubMed:26823428) . Mediates the transport of chloride ions across the cell membrane (PubMed:10792060, PubMed:11524016, PubMed:11707463, PubMed:12519745, PubMed:15010471, PubMed:12588899, PubMed:17036051, PubMed:19398555, PubMed:19621064, PubMed:22178883, PubMed:25330774, PubMed:1712898, PubMed:8910473, PubMed:9804160, PubMed:12529365, PubMed:17182731, PubMed:26846474, PubMed:28087700) . Channel activity is coupled to ATP hydrolysis (PubMed:8910473) . The ion channel is also permeable to HCO (3-) ; selectivity depends on the extracellular chloride concentration (PubMed:15010471, PubMed:19019741) . Exerts its function also by modulating the activity of other ion channels and transporters (PubMed:12403779, PubMed:22178883, PubMed:22121115, PubMed:27941075) . Plays an important role in airway fluid homeostasis (PubMed:16645176, PubMed:19621064, PubMed:26823428) . Contributes to the regulation of the pH and the ion content of the airway surface fluid layer and thereby plays an important role in defense against pathogens (PubMed:14668433, PubMed:16645176, PubMed:26823428) . Modulates the activity of the epithelial sodium channel (ENaC) complex, in part by regulating the cell surface expression of the ENaC complex (PubMed:17434346, PubMed:27941075, PubMed:17182731) . Inhibits the activity of the ENaC channel containing subunits SCNN1A, SCNN1B and SCNN1G (PubMed:17182731) . Inhibits the activity of the ENaC channel containing subunits SCNN1D, SCNN1B and SCNN1G, but not of the ENaC channel containing subunits SCNN1A, SCNN1B and SCNN1G (PubMed:27941075) . May regulate bicarbonate secretion and salvage in epithelial cells by regulating the transporter SLC4A7 (PubMed:12403779) . Can inhibit the chloride channel activity of ANO1 (PubMed:22178883) . Plays a role in the chloride and bicarbonate homeostasis during sperm epididymal maturation and capacitation (PubMed:19923167, PubMed:27714810) .