Allosteric modulation balances thermodynamic stability and restores function of ΔF508 CFTR.
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Mechanisms of CFTR Folding at the Endoplasmic ReticulumThe silent codon change I507-ATC->ATT contributes to the severity of the ΔF508 CFTR channel dysfunctionImpact of the F508del mutation on ovine CFTR, a Cl- channel with enhanced conductance and ATP-dependent gating.Decoding F508del misfolding in cystic fibrosis.The N-terminal domain allosterically regulates cleavage and activation of the epithelial sodium channel.Cystic fibrosis transmembrane conductance regulator (CFTR) potentiators protect G551D but not ΔF508 CFTR from thermal instability.Potentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosis.Rational Coupled Dynamics Network Manipulation Rescues Disease-Relevant Mutant Cystic Fibrosis Transmembrane Conductance Regulator.Structure-based rational design to enhance the solubility and thermostability of a bacterial laccase Lac15Full-open and closed CFTR channels, with lateral tunnels from the cytoplasm and an alternative position of the F508 region, as revealed by molecular dynamics.Thermal instability of ΔF508 cystic fibrosis transmembrane conductance regulator (CFTR) channel function: protection by single suppressor mutations and inhibiting channel activityMolecular modelling and molecular dynamics of CFTR.Deletion of Phenylalanine 508 in the First Nucleotide-binding Domain of the Cystic Fibrosis Transmembrane Conductance Regulator Increases Conformational Exchange and Inhibits Dimerization.Correctors of ΔF508 CFTR restore global conformational maturation without thermally stabilizing the mutant protein.Restoration of NBD1 thermal stability is necessary and sufficient to correct ∆F508 CFTR folding and assemblyDynamics intrinsic to cystic fibrosis transmembrane conductance regulator function and stabilityMechanism-based corrector combination restores ΔF508-CFTR folding and function.Functional Rescue of F508del-CFTR Using Small Molecule CorrectorsCystic fibrosis transmembrane conductance regulator (ABCC7) structureBreakthrough therapies: Cystic fibrosis (CF) potentiators and correctors.Two Small Molecules Restore Stability to a Subpopulation of the Cystic Fibrosis Transmembrane Conductance Regulator with the Predominant Disease-causing Mutation.Thermal stability of purified and reconstituted CFTR in a locked open channel conformation.The major cystic fibrosis causing mutation exhibits defective propensity for phosphorylation.Revertant mutants modify, but do not rescue, the gating defect of the cystic fibrosis mutant G551D-CFTR.Thermal unfolding simulations of NBD1 domain variants reveal structural motifs associated with the impaired folding of F508del-CFTR.Functional reconstitution and channel activity measurements of purified wildtype and mutant CFTR protein.The holy grail of cystic fibrosis research: pharmacological repair of the F508del-CFTR mutation.Membrane protein stability can be compromised by detergent interactions with the extramembranous soluble domainsEnhanced thermal stability of Pseudomonas aeruginosa lipoxygenase through modification of two highly flexible regions.Substitution of Yor1p NBD1 residues improves the thermal stability of Human Cystic Fibrosis Transmembrane Conductance Regulator.A role for the cystic fibrosis transmembrane conductance regulator in the nitric oxide-dependent release of Cl- from acidic organelles in amacrine cells.Mapping allosteric linkage to channel gating of extracellular domains in the human epithelial sodium channel.CFTR structure: lassoing cystic fibrosis.Sites associated with Kalydeco binding on human Cystic Fibrosis Transmembrane Conductance Regulator revealed by Hydrogen/Deuterium Exchange.Impact of the cystic fibrosis mutation F508del-CFTR on renal cyst formation and growthGraph-Theoretic Models of Mutations in the Nucleotide Binding Domain 1 of the Cystic Fibrosis Transmembrane Conductance Regulator
P2860
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P2860
Allosteric modulation balances thermodynamic stability and restores function of ΔF508 CFTR.
description
2012 nî lūn-bûn
@nan
2012年の論文
@ja
2012年学术文章
@wuu
2012年学术文章
@zh-cn
2012年学术文章
@zh-hans
2012年学术文章
@zh-my
2012年学术文章
@zh-sg
2012年學術文章
@yue
2012年學術文章
@zh
2012年學術文章
@zh-hant
name
Allosteric modulation balances ...... stores function of ΔF508 CFTR.
@en
Allosteric modulation balances ...... stores function of ΔF508 CFTR.
@nl
type
label
Allosteric modulation balances ...... stores function of ΔF508 CFTR.
@en
Allosteric modulation balances ...... stores function of ΔF508 CFTR.
@nl
prefLabel
Allosteric modulation balances ...... stores function of ΔF508 CFTR.
@en
Allosteric modulation balances ...... stores function of ΔF508 CFTR.
@nl
P2093
P2860
P1476
Allosteric modulation balances ...... stores function of ΔF508 CFTR.
@en
P2093
Alexey E Alekseev
Andrei A Aleksandrov
John R Riordan
Liying Cui
Luba A Aleksandrov
Martina Gentzsch
Pradeep Kota
Santiago Reyes
Tim Jensen
P2860
P356
10.1016/J.JMB.2012.03.001
P407
P577
2012-03-08T00:00:00Z