High-throughput screening identifies FAU protein as a regulator of mutant cystic fibrosis transmembrane conductance regulator channel
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چکیده
منابع مشابه
Cystic Fibrosis Transmembrane Conductance Regulator
Description The cystic fibrosis transmembrane regulator (CFTR) gene codes for the CFTR protein; a chloride channel protein that helps in the transportation of chloride ions and water molecules across the cell membranes of lungs, liver, pancreas, and skin. CFTR is a member of the ATP-binding cassette family of membrane transport proteins, but appears to be unique within this family by functionin...
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The cystic fibrosis transmembrane conductance regulator (CFTR) Cl channel exhibits lyotropic anion selectivity. Anions that are more readily dehydrated than Cl exhibit permeability ratios (P(S)/P(Cl)) greater than unity and also bind more tightly in the channel. We compared the selectivity of CFTR to that of a synthetic anion-selective membrane [poly(vinyl chloride)-tridodecylmethylammonium chl...
متن کاملLocalization of Cystic Fibrosis Transmembrane Conductance Regulator
We have used in situ hybridization to localize expression of the cystic fibrosis transmembrane conductance regulator (CFTR) gene in the human gastrointestinal tract and associated organs. The stomach exhibits a low level ofCFTR expression throughout gastric mucosa. In the small intestine, expression is relatively high in the mucosal epithelium, with a decreasing gradient of expression along the...
متن کاملChloride channel and chloride conductance regulator domains of CFTR, the cystic fibrosis transmembrane conductance regulator.
CFTR is a cyclic AMP (cAMP)-activated chloride (Cl-) channel and a regulator of outwardly rectifying Cl- channels (ORCCs) in airway epithelia. CFTR regulates ORCCs by facilitating the release of ATP out of cells. Once released from cells, ATP stimulates ORCCs by means of a purinergic receptor. To define the domains of CFTR important for Cl- channel function and/or ORCC regulator function, mutan...
متن کاملCystic Fibrosis Transmembrane Conductance Regulator (CFTR) Gene
The Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene was identified in 1989 by geneticist Lap-Chee Tsui and his research team as the gene associated with cystic fibrosis [4] (CF). Tsui?s research pinpointed the gene, some mutations to which cause CF, and it revealed the underlying disease mechanism. The CFTR gene encodes a protein in cell membranes in epithelial tissues and affec...
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ژورنال
عنوان ژورنال: Journal of Biological Chemistry
سال: 2018
ISSN: 0021-9258
DOI: 10.1074/jbc.m117.816595