CFTR
Reaktivität: Human
ISt, IHC (f)
Wirt: Maus
Monoclonal
CFTR-1785
unconjugated
Applikationshinweise
ELISA: 1: 1000approx. 1: 5000. IHC: 1: 50approx. 1: 200. Other applications not tested. Optimal dilutions are dependent on conditions and should be determined by the user.
CFTR, for cystic fibrosis transmembrane conductance regulator, is a cyclic adenosine monophosphate (cAMP)-regulated chloride channel protein. CFTR belongs to the MDR subfamily within the ATP-binding transport protein family. It has two transmembrane domains (TMDs), two nucleotide binding domains (NBDs) and one regulatory domain. Mutations of CFTR are associated with cystic fibrosis (CF), a disease characterized by chronic bronchopulmonary disease, elevated sweat electrolytes and insufficient pancreatic function. CFTR mutations can also result in congenital bilateral absence of vas deferens (CBAVD), a form 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 question of why this genetic disease remains as common as it is. One possible explanation is that Salmonella typhi has been shown to use CFTR to enter intestinal epithelial cells and that delta F508 heterozygote and homozygote mice showed 86 % and 100 % reductions in S. typhi intestinal submucosal uptake.Synonyms: ATP-binding cassette transporter sub-family C member 7, Channel conductance-controlling ATPase, Cystic fibrosis transmembrane conductance regulator, cAMP-dependent chloride channel