Specificity
Recognizes a protein of 165-170kDa, identified as cystic fibrosis transmembrane conductance regulator (CFTR). CFTR 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-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.