|Statement||by Catherine M. O"Reilly.|
|Contributions||University College Dublin. Department of Pharmacology.|
|The Physical Object|
|Pagination||xvi,183p., p. of plates :|
|Number of Pages||183|
Cystic fibrosis (CF) is a fatal genetic disease caused by a defect in cyclic AMP dependent Cl − transport in exocrine epithelia (Quinton, ). It has been shown that mutation of the CF gene leads to absence, dysregulation or dysfunction of the Cl − channel encoded by the gene, the cystic fibrosis transmembrane conductance regulator (CFTR) (Welsh & Smith, ).Cited by: Loss of CFTR has profound effects on chloride transport both directly and indirectly through these pathways, compounding the defects in epithelial chloride transport that result from loss of CFTR. Another important cellular effect of CFTR is volume regulation through the Ca 2+-dependent potassium channel, KCNN4. During regulatory volume decrease (RVD), anion and cation channels are . Amphotropic retroviruses were used to transduce a functional cystic fibrosis transmembrane conductance regulator (CFTR) cDNA into CFPAC-1, a pancreatic adenocarcinoma cell line derived from a. Transport of Na + and Cl − is a major task of epithelial cells and requires regulation of both Cl − and Na + conductive pathways (Greger, Kunzelmann & Gerlach, ).Both types of conductance pathways, the cystic fibrosis transmembrane conductance regulator (CFTR) and epithelial Na + channels (ENaC) (Riordan et al. ; Canessa et al. ), are co‐expressed in apical .
The cystic fibrosis transmembrane conductance regulator (CFTR) is defective in cystic fibrosis (CF). This protein is a channel that sits on the surface of cells and transports chloride and other molecules, such as bicarbonate. The gene that encodes the CFTR protein, which is also called CFTR, is located on chromosome 7. Mutations in this gene lead to CF. CF is an autosomal recessive disorder of chloride transport resulting from mutations in the cystic fibrosis transmembrane regulator (CFTR) gene. The defect in the CFTR protein results in abnormal chloride transport, leading to abnormally viscous secretions eventually causing organ damage. Catherine M O'Reilly, Anne M O'Farrell, Michael P Ryan, Purinoceptor activation of chloride transport in cystic fibrosis and CFTR‐transfected pancreatic cell lines, British Journal of Pharmacology, /, , 8, (), (). In healthy cells CFTR acts as a chloride channel and a regulator of sodium, chloride and bicarbonate transport. The widespread presence of CFTR throughout the body (lungs, salivary glands, pancreas, liver, kidneys, sweat ducts and reproductive tract) helps to explain why CF is a multisystem condition affecting many organs (figure 1).
Cystic fibrosis transmembrane conductance regulator (CFTR) is a membrane protein and chloride channel in vertebrates that is encoded by the CFTR gene. The CFTR gene codes for an ABC transporter -class ion channel protein that conducts chloride ions across epithelial cell membranes. Purinoceptor activation of chloride transport in cystic fibrosis and CFTR-transfected pancreatic cell lines British Journal of Pharmacology, Vol. , No. 8 Characterization of PKA isoforms and kinase-dependent activation of chloride secretion in T84 cells. A recent review describes the evidence that ASL pH is controlled by bicarbonate secretion via CFTR and other secreted proteins. 2 CFTR in airway cells derived from a lung cancer cell line (Calu‐3) will secrete either bicarbonate or chloride, depending on the activity of basolateral membrane cotransporters. 3 In whole animal systems (small. Trapnell BC, Zeitlin PL, Chu C-S, Yoshimura K, Nakamura H, Guggino WB, Bargon J, Banks TC, Dalemans W, Pavirani A, Lecocq J-P, Crystal RG. Down-regulation of cystic fibrosis gene mRNA transcript levels and induction of the cystic fibrosis chloride secretory phenotype in epithelial cells by phorbol ester. J Biol Chem ; –