The Cystic Fibrosis Transmembrane Conductance Regulator
Kevin L. Kirk, David C. Dawson
Springer Science & Business Media, Oct 31, 2003 - Science - 215 pages
The CFTR chloride channel is one of the most well studied transport proteins in biology. Yet there remain many mysteries about the functional properties and biological roles of this ABC transporter. The Cystic Fibrosis Transmembrane Conductance Regulator addresses a select series of `hot' topics that relate to the function of CFTR, and the links between CFTR dysfunction and human disease (i.e., cystic fibrosis). The timeliness of these topics distinguishes this collection from previous volumes of this type. Given the general interest in CFTR, this collection will appeal to a broad readership with interests in CFTR, cystic fibrosis, ion channels and ABC transporters.
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absorption acid airway epithelial cells airway surface amiloride amiloride sensitive amino anion apical membrane assays bacterial basolateral binding Biochem Biol Chem cAMP cell lines CF airway CF patients CFTR channels CFTR chloride channel CFTR expression CFTR gene CFTR-mediated channel activity chloride channel Cl channels Clin Invest colon compounds concentration cultures currents cystic fibrosis transmembrane Defective disease effects ENaC epithelial cells epithelial sodium channel epithelium exon fibrosis transmembrane conductance Gen Physiol glands human airway increased infection inflammatory response inhibition of ENaC interactions intracellular ion channels lung mechanism mediated mice mouse mucus mutations nasal Natl Acad Sci neutrophils NF-kB non-CF normal oocytes PDZ domain permeability phosphorylation plasma membrane pore potential PP2A PP2C Proc Natl Acad protein kinase protein phosphatase Pseudomonas aeruginosa receptor regulator CFTR regulatory role secretion stimulation structure studies subunit syntaxin syntaxin 1A tion tissues transepithelial transmembrane conductance regulator transport vectors vitro vivo wild-type Xenopus oocytes