G551D and G1349D, two CF-associated mutations in the signature sequences of CFTR, exhibit distinct gating defects
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Targeted therapies to improve CFTR function in cystic fibrosisAllosteric coupling between the intracellular coupling helix 4 and regulatory sites of the first nucleotide-binding domain of CFTRObligate coupling of CFTR pore opening to tight nucleotide-binding domain dimerizationCurcumin and genistein: the combined effects on disease-associated CFTR mutants and their clinical implicationsStabilization of Nucleotide Binding Domain Dimers Rescues ABCC6 Mutants Associated with Pseudoxanthoma Elasticum.A little CFTR goes a long way: CFTR-dependent sweat secretion from G551D and R117H-5T cystic fibrosis subjects taking ivacaftorFolding and rescue of a cystic fibrosis transmembrane conductance regulator trafficking mutant identified using human-murine chimeric proteinsATP-independent CFTR channel gating and allosteric modulation by phosphorylation.Stable ATP binding mediated by a partial NBD dimer of the CFTR chloride channelPotentiation of disease-associated cystic fibrosis transmembrane conductance regulator mutants by hydrolyzable ATP analogs.Conserved allosteric hot spots in the transmembrane domains of cystic fibrosis transmembrane conductance regulator (CFTR) channels and multidrug resistance protein (MRP) pumps.Cystic fibrosis transmembrane conductance regulator (CFTR) potentiator VX-770 (ivacaftor) opens the defective channel gate of mutant CFTR in a phosphorylation-dependent but ATP-independent manner.Dual roles of the sixth transmembrane segment of the CFTR chloride channel in gating and permeation.A single amino acid substitution in CFTR converts ATP to an inhibitory ligand.Cataract-causing defect of a mutant γ-crystallin proceeds through an aggregation pathway which bypasses recognition by the α-crystallin chaperone.Proteomic identification of calumenin as a G551D-CFTR associated protein.Optimization of the degenerated interfacial ATP binding site improves the function of disease-related mutant cystic fibrosis transmembrane conductance regulator (CFTR) channels.On the mechanism of CFTR inhibition by a thiazolidinone derivative.State-dependent regulation of cystic fibrosis transmembrane conductance regulator (CFTR) gating by a high affinity Fe3+ bridge between the regulatory domain and cytoplasmic loop 3.The inhibition mechanism of non-phosphorylated Ser768 in the regulatory domain of cystic fibrosis transmembrane conductance regulatorPotentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosis.Dual activity of aminoarylthiazoles on the trafficking and gating defects of the cystic fibrosis transmembrane conductance regulator chloride channel caused by cystic fibrosis mutations.Localizing a gate in CFTR.Genotype-phenotype correlation in cystic fibrosis patients bearing [H939R;H949L] alleleBiophysical characterisation of calumenin as a charged F508del-CFTR folding modulatorImproved fluorescence assays to measure the defects associated with F508del-CFTR allow identification of new active compounds.The CFTR ion channel: gating, regulation, and anion permeationLong-range coupling between the extracellular gates and the intracellular ATP binding domains of multidrug resistance protein pumps and cystic fibrosis transmembrane conductance regulator channelsFrom CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations.Vx-770 potentiates CFTR function by promoting decoupling between the gating cycle and ATP hydrolysis cycle.Converting nonhydrolyzable nucleotides to strong cystic fibrosis transmembrane conductance regulator (CFTR) agonists by gain of function (GOF) mutationsThree-dimensional reconstruction of human cystic fibrosis transmembrane conductance regulator chloride channel revealed an ellipsoidal structure with orifices beneath the putative transmembrane domainCombination therapy with cystic fibrosis transmembrane conductance regulator modulators augment the airway functional microanatomy.Mutations at the signature sequence of CFTR create a Cd(2+)-gated chloride channel.Nonequilibrium gating of CFTR on an equilibrium theme.Application of high-resolution single-channel recording to functional studies of cystic fibrosis mutants.CLC-0 and CFTR: chloride channels evolved from transporters.State-dependent modulation of CFTR gating by pyrophosphate.Revisiting the role of cystic fibrosis transmembrane conductance regulator and counterion permeability in the pH regulation of endocytic organelles.Mutation-specific potency and efficacy of cystic fibrosis transmembrane conductance regulator chloride channel potentiators.
P2860
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P2860
G551D and G1349D, two CF-associated mutations in the signature sequences of CFTR, exhibit distinct gating defects
description
2007 nî lūn-bûn
@nan
2007年の論文
@ja
2007年学术文章
@wuu
2007年学术文章
@zh-cn
2007年学术文章
@zh-hans
2007年学术文章
@zh-my
2007年学术文章
@zh-sg
2007年學術文章
@yue
2007年學術文章
@zh
2007年學術文章
@zh-hant
name
G551D and G1349D, two CF-assoc ...... xhibit distinct gating defects
@ast
G551D and G1349D, two CF-assoc ...... xhibit distinct gating defects
@en
type
label
G551D and G1349D, two CF-assoc ...... xhibit distinct gating defects
@ast
G551D and G1349D, two CF-assoc ...... xhibit distinct gating defects
@en
prefLabel
G551D and G1349D, two CF-assoc ...... xhibit distinct gating defects
@ast
G551D and G1349D, two CF-assoc ...... xhibit distinct gating defects
@en
P2093
P2860
P356
P1476
G551D and G1349D, two CF-assoc ...... xhibit distinct gating defects
@en
P2093
Silvia G Bompadre
Tzyh-Chang Hwang
Yoshiro Sohma
P2860
P304
P356
10.1085/JGP.200609667
P577
2007-03-12T00:00:00Z