The Cystic Fibrosis Transmembrane Conductance Regulator
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 activity addition aeruginosa altered anion apical membrane assays associated bacterial binding Biochem Biol Chem block CAMP caused CFTR channels CFTR gene changes channel activity chloride channel compared complex compounds concentration containing cultures currents cystic fibrosis transmembrane cytoplasmic Defective dependent direct disease domain effects ENaC energy epithelial cells epithelium et al evidence expression fibrosis transmembrane conductance function gating gene glands human important increased infection inflammatory inhibition interactions intracellular involved levels lung measurements mechanism mediated membrane mice mutations nasal Nature neutrophils normal observed patch permeability phosphatase Physiol plasma membrane pore possible potential present production properties protein protein kinase recent receptor reduced region regulatory reported Respir response role secretion selectivity sensitive sequence showed single sodium specific stimulation structure studies suggested surface tion tissues transmembrane conductance regulator transport vivo Xenopus oocytes
Página 199 - Cooke, RE, A test for concentration of electrolytes in sweat in cystic fibrosis of the pancreas utilizing pilocarpine by iontophoresis, Pediatrics, 23, 545, 1959.