PA1-935 detects cystic fibrosis transmembrane conductance factor (CFTR) from cells overexpressing the human protein.
PA1-935 has been successfully used in immunocytochemistry procedures. Immunocytochemical staining of HEK293 cells overexpressing human CFTR with this antibody results in staining primarily of the plasma membrane.
PA1-935 immunizing peptide corresponds to amino acid residues 103-117 from human CFTR protein. This sequence is completely conserved between human, rabbit, and monkey and there is a one amino acid substitution in rat, bovine, and sheep.
Cystic Fibrosis (CF) is a common lethal genetic disease caused by mutations of the gene coding for the cystic fibrosis transmembrane conductance factor, a cAMP regulated chloride channel. Approximately 70% of all CF cases share the deletion of a phenylalanine at position 508 (delta F508) which results in abnormal chloride transport.
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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Protein Aliases: ATP-binding cassette sub-family C member 7; cAMP-dependent chloride channel; CFTR; Channel conductance-controlling ATPase; Cystic Fibrosis Transmembrane Conductance Regulator; cystic fibrosis transmembrane conductance regulator (ATP-binding cassette sub-family C, member 7)
Gene Aliases: ABC35; ABCC7; CF; CFTR; CFTR/MRP; dJ760C5.1; MRP7; TNR-CFTR
UniProt ID: (Human) P13569
Entrez Gene ID: (Human) 1080