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1 CFTR activators have potential therapeutic indications i
2 CFTR channel gating is strictly coupled to phosphorylati
3 CFTR dysfunction affects innate immune pathways, generat
4 CFTR dysfunction mainly affects epithelial cells, althou
5 CFTR is a major prosecretory chloride channel at the ocu
6 CFTR is expressed in various cell types, including leuko
7 CFTR modulator therapy with tezacaftor-ivacaftor or ivac
8 CFTR sequencing demonstrated that she is a carrier for a
9 CFTR, the chloride channel mutated in cystic fibrosis (C
10 CFTR, the cystic fibrosis (CF) gene, encodes for the CFT
11 definition of the druggability of the 14-3-3-CFTR interface might offer an approach for cystic fibros
12 confirmed diagnosis of cystic fibrosis and a CFTR gating mutation on at least one allele from 15 hosp
13 eterozygous for the Phe508del mutation and a CFTR mutation associated with residual CFTR function.
17 r alone or in combination with tezacaftor, a CFTR corrector, in 248 patients 12 years of age or older
18 -) currents, but unexpectedly also abrogates CFTR-mediated Cl(-) secretion and completely abolishes c
19 he most potent compound, 12, fully activated CFTR chloride conductance with EC50 approximately 30 nM,
20 3,5-triazine CFTRact-K089 (1) that activated CFTR with EC50 approximately 250 nM, which when delivere
22 ata provide insights into how loss of active CFTR at the membrane can have additional consequences be
23 ane Conductance Regulator (CFTR) gene affect CFTR protein biogenesis or its function as a chloride ch
24 t the C-terminal PDZ-domain of both A2BR and CFTR were crucial for this interaction, and that replaci
25 independent screens, firefly luciferase and CFTR-mediated transepithelial chloride conductance assay
28 gnificant ABCC transporters (MRP1, SUR1, and CFTR), determined by using single-particle cryo-electron
30 e proteostasis of other membrane proteins as CFTR and anthrax toxin receptor 2, two poor folders invo
32 o better understand the relationship between CFTR activity, airway microbiology and inflammation, and
34 l information on the unique ABC ion channel, CFTR, hinders elucidation of its functional mechanism an
36 ulator (CFTR) gene, is capable of correcting CFTR-dependent chloride transport in cystic fibrosis hum
37 otal enhancer element dramatically decreases CFTR expression, but has minor effect on its 3D structur
38 ack of an effect of C4 on I507-ATC DeltaF508 CFTR, but its additive effect in combination with VX-809
42 ansmembrane conductance regulator (DeltaF508 CFTR), the most frequent disease-associated mutant of CF
44 /potentiator strategy, as used for DeltaF508-CFTR, to produce functional rescue of the truncated tran
45 the loss-of-function phenotype of DeltaF508-CFTR suggest that the ribosomal stalk modulates the fold
49 these candidate genes enhanced the DeltaF508-CFTR functional expression at the apical PM in human CF
53 mparison with MRP1, a feature distinguishing CFTR from all other ABC transporters is the helix-loop t
54 that cigarette smoke not only downregulates CFTR activity but also inhibits BK channel function, the
59 orrector-potentiator therapy, which enhances CFTR transport to the membrane, have increased PTEN amou
60 -channel recording, whereas to assess entire CFTR populations, we used purified CFTR proteins and mac
61 ht produce a greater benefit than expressing CFTR at wild-type levels when targeting small fractions
62 nsitive mutant cystic fibrosis channel (F508-CFTR) at the plasma membrane and after reconstitution in
63 concomitant functional inactivation of F508-CFTR are partially suppressed by constitutive activity o
64 contribute to functional maintenance of F508-CFTR by reshaping the conformational energetics of its f
65 SUMOylation, to selectively degrade F508del CFTR via the SUMO-targeted ubiquitin E3 ligase, RNF4 (RI
69 h cystic fibrosis and homozygous for F508del-CFTR, but it has not been assessed in younger patients.
73 ated autophagy in primary homozygous F508del-CFTR human bronchial epithelial (hBE) cells at submicrom
75 onic co-incubation greatly increased F508del-CFTR channel activity and temporal stability in most, bu
78 ally stabilized purified full-length F508del-CFTR and slightly delayed deactivation of individual F50
81 tability in a small subpopulation of F508del-CFTR Cl(-) channels but that the majority remain destabi
84 Chronic (prolonged) co-incubation of F508del-CFTR-expressing cells with lumacaftor and ivacaftor deac
85 hose silencing significantly rescued F508del-CFTR activity, as indicated by enhanced anion transport
86 7 degrees C, low temperature-rescued F508del-CFTR more rapidly lost function in cell-free membrane pa
88 nd the characterization of the small F508del-CFTR subpopulation might be crucial for CF therapy devel
89 ultaneously act as correctors of the F508del-CFTR folding defect and as broad-spectrum antivirals aga
93 To identify proteins associated with F508del-CFTR processing, we used a high-throughput functional as
96 side in NBD2, and the domain is critical for CFTR function, because channel gating involves NBD1/NBD2
99 cidated structure-activity relationships for CFTR activation and identified substantially more potent
101 ummary, we have described a direct link from CFTR to Ezrin to PI3K/AKT signaling that is disrupted in
102 rously validated candidates using functional CFTR maturation and electrolyte transport assays in pola
103 ion in people with cystic fibrosis and G551D-CFTR mutations but does not reduce density of bacteria o
110 a 3.9 A structure of dephosphorylated human CFTR without nucleotides, determined by electron cryomic
118 Riquelme et al. (2017) propose that improved CFTR trafficking could enhance P. aeruginosa clearance t
120 (-) transport given that neither a change in CFTR-dependent HCO3 (-) efflux nor Na(+) /HCO3 (-) cotra
121 turbing the gating conformational changes in CFTR's transmembrane domains (TMDs) without altering the
125 osine- and forskolin-stimulated increases in CFTR-dependent transepithelial short-circuit current, in
127 f 21 (86%) known splice-altering variants in CFTR, a well-studied gene whose loss-of-function variant
128 absorption, while anion channels, including CFTR and Ca(2+)-activated chloride channels mediate anio
129 the apical side of cholangiocytes, including CFTR and SLC5A1, as well as reduced expression of IGF1.
134 is not membrane delimited and that inhibited CFTR channels remain at the cell membrane, indicative of
135 ido-pyrrolo-oxazinedione (R)-BPO-27 inhibits CFTR chloride conductance with low-nanomolar potency.
136 sm by which sphingomyelin catalysis inhibits CFTR is not known but evidence suggests that it occurs i
137 ther, these data suggest that SMase inhibits CFTR channel function by locking channels into a closed
143 e have previously shown that plasma membrane CFTR increases the surface density of the adenosine 2B r
144 el, results in the production of a misfolded CFTR protein, which has residual channel activity but is
152 centration provides an in vivo assessment of CFTR function, but it is unknown the degree to which CFT
153 To understand better the kinetic basis of CFTR intraburst gating, we investigated the single-chann
158 ed on the molecular phenotypic complexity of CFTR mutants and their susceptibility to pharmacotherapy
160 By analyzing the sigmoid time course of CFTR current activation, we propose that PKA phosphoryla
162 Phosphorylation of the regulatory domain of CFTR by protein kinase A (PKA) is required for its chann
163 ce between the catalytic and pore domains of CFTR and that this modification facilitates CFTR channel
164 tion of channel openings, the dysfunction of CFTR in CF and the action of drugs that repair CFTR gati
170 d inflammation may progress independently of CFTR activity once cystic fibrosis lung disease is estab
172 Importantly, pharmacological inhibition of CFTR abrogated enteroid fluid secretion, providing proof
175 e most frequent disease-associated mutant of CFTR, may affect protein biogenesis, structure, and func
178 ing the HCO3(-) /Cl(-) permeability ratio of CFTR from 0.4 to 1.0 had little impact upon either the s
179 interaction between the regulatory region of CFTR and calmodulin, the major calcium signaling molecul
181 her, our data suggest that the regulation of CFTR intraburst gating is distinct from the ATP-dependen
185 ta highlight the critical regulatory role of CFTR in integrin activation by chemoattractants in monoc
187 represent subtle changes in the structure of CFTR that are regulated by intracellular pH, in part, at
188 Intrinsic tryptophan fluorescence studies of CFTR showed that phosphorylation reduced iodide-mediated
190 Our results support the potential utility of CFTR-targeted activators as a novel prosecretory treatme
194 bed indirect effects of calcium signaling on CFTR or other calcium-activated chloride channels; here,
195 results highlight the importance of not only CFTR but also BK channel function in maintaining ASL hom
197 and apical Cl(-) and HCO3(-) permeabilities (CFTR), and reducing the activity of the basolateral Cl(-
200 the single-channel conductance (g) in R117H-CFTR, but found a approximately 13-fold lower open proba
201 rate that a synergistic improvement of R117H-CFTR function can be accomplished with a combined regime
202 acterizations of the gating defects of R117H-CFTR led to the conclusion that the mutation decreases P
204 se reagents potentiate synergistically R117H-CFTR gating to a level that allows accurate assessments
206 ibrosis transmembrane conductance regulator (CFTR or ABCC7; i.e., G551D, S1251N, and G1349D), that we
207 ibrosis transmembrane conductance regulator (CFTR) activator with an EC50 of approximately 200 nM and
208 ibrosis transmembrane conductance regulator (CFTR) activity and lung function in people with cystic f
210 ibrosis transmembrane conductance regulator (CFTR) and large-conductance, Ca(2+)-activated, and volta
211 ibrosis transmembrane conductance regulator (CFTR) anion channel causes misfolding and premature degr
212 ibrosis transmembrane conductance regulator (CFTR) anion channels and solute carrier family 26 member
213 ibrosis transmembrane conductance regulator (CFTR) channel, which can result in chronic lung disease.
215 ibrosis transmembrane conductance regulator (CFTR) chloride channel, leading to defective apical chlo
217 ibrosis transmembrane conductance regulator (CFTR) combined with hyperactivation of the epithelial so
218 ibrosis transmembrane conductance regulator (CFTR) first cytosolic loop (CL1) and nucleotide binding
219 ibrosis transmembrane conductance regulator (CFTR) folding defect responsible for >90% of CF cases.
220 ibrosis Transmembrane Conductance Regulator (CFTR) gene affect CFTR protein biogenesis or its functio
221 ibrosis transmembrane conductance regulator (CFTR) gene cause cystic fibrosis (CF), but are not good
222 ibrosis transmembrane conductance regulator (CFTR) gene, is capable of correcting CFTR-dependent chlo
223 ibrosis transmembrane conductance regulator (CFTR) has lagged behind research into the NBD1 domain, i
224 ibrosis transmembrane conductance regulator (CFTR) have been described that confer a range of molecul
225 ibrosis transmembrane conductance regulator (CFTR) have blunted induction of PI3K/AKT signaling in re
226 ibrosis transmembrane conductance regulator (CFTR) in placebo-controlled studies and patients aged 6-
227 the CF transmembrane conductance regulator (CFTR) interacted directly and this interaction was neces
228 ibrosis transmembrane conductance regulator (CFTR) is a multidomain membrane protein that functions a
229 ibrosis transmembrane conductance regulator (CFTR) is an anion channel evolved from an ATP-binding ca
230 ibrosis transmembrane conductance regulator (CFTR) is an ATP-binding cassette (ABC) transporter that
231 ibrosis transmembrane conductance regulator (CFTR) is an ATP-gated Cl(-) channel defective in the gen
232 ibrosis transmembrane conductance regulator (CFTR) is an epithelial anion channel and a key regulator
233 ibrosis transmembrane conductance regulator (CFTR) is key for the optimization of therapeutics as wel
235 ibrosis Transmembrane Conductance Regulator (CFTR) is the secretory chloride/bicarbonate channel in a
236 ibrosis transmembrane conductance regulator (CFTR) modulators tezacaftor (VX-661) and ivacaftor (VX-7
237 ibrosis transmembrane conductance regulator (CFTR) mutation in humans, DeltaF508, show increased morb
238 ibrosis transmembrane conductance regulator (CFTR) protein levels, and transepithelial resistance.
239 is (CF) transmembrane conductance regulator (CFTR) regulates bile secretion and other functions at th
240 ibrosis transmembrane conductance regulator (CFTR) that compromise its chloride channel activity.
241 ibrosis transmembrane conductance regulator (CFTR) that reduces Pseudomonas aeruginosa culture positi
242 ibrosis transmembrane conductance regulator (CFTR) W1282X PTC (a UGA codon) in the context of its thr
243 ibrosis transmembrane conductance regulator (CFTR), a 25% reduction of the single-channel conductance
244 ibrosis transmembrane conductance regulator (CFTR), F508del, is initiated by binding of the small hea
245 ibrosis transmembrane conductance regulator (CFTR), leading to detrimental changes to protein stabili
246 ibrosis transmembrane conductance regulator (CFTR), which is defective in the genetic disease cystic
247 g in CF transmembrane conductance regulator (CFTR)-deficient organoids and by nasal potential differe
256 oke-induced channel dysfunction reveals that CFTR activity is downregulated via Smad3 signalling wher
257 evented actin rearrangement, suggesting that CFTR insertion in the plasma membrane results in local r
258 of CFTR in the enteric ganglia suggests that CFTR may play a role in the physiology of the innervatio
264 e cystic fibrosis (CF) gene, encodes for the CFTR protein that plays an essential role in anion regul
265 ystic fibrosis is caused by mutations in the CFTR chloride channel, leading to reduced airway surface
266 recessive disease caused by mutations in the CFTR gene that lead to progressive respiratory decline.
271 ns show residual function and respond to the CFTR potentiator ivacaftor in vitro, whereas ivacaftor a
272 the mutation K1250A or pretreating with the CFTR potentiator VX-770 (Ivacaftor) imparted resistance
274 tics stimulates lung fluid secretion through CFTR, an effect which in humans, but not mice, was also
279 The major contribution (>/=90%) to the total CFTR-related ATP hydrolysis rate is due to phosphorylati
280 t rAAV-mediated gene transfer of a truncated CFTR functionally rescues the CF phenotype across the na
282 al effects of this competition for wild-type CFTR and the major F508del mutant, hinting at potential
283 onfirmed that purified full-length wild-type CFTR is folded and structurally responsive to phosphoryl
292 nt data are consistent with a model in which CFTR is in a closed conformation with two ATPs bound.
293 ction, but it is unknown the degree to which CFTR mutations account for sweat chloride variation.
295 Across the tested CF population as a whole, CFTR gene mutations were found to be the primary determi
296 est that PTC suppression in combination with CFTR modulators may be beneficial for the treatment of C
299 Here, we present the structure of zebrafish CFTR in the phosphorylated, ATP-bound conformation, dete
300 ce of this human CFTR structure to zebrafish CFTR under identical conditions reinforces its relevance
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