The role of cystic fibrosis transmembrane conductance regulator phenylalanine 508 side chain in ion channel gating.
about
Allosteric coupling between the intracellular coupling helix 4 and regulatory sites of the first nucleotide-binding domain of CFTRThe silent codon change I507-ATC->ATT contributes to the severity of the ΔF508 CFTR channel dysfunctionGenetic selection for a highly functional cysteine-less membrane protein using site saturation mutagenesisRestoration of domain folding and interdomain assembly by second-site suppressors of the DeltaF508 mutation in CFTRDual roles of the sixth transmembrane segment of the CFTR chloride channel in gating and permeation.Cysteine-scanning analysis of the nucleobase-ascorbate transporter signature motif in YgfO permease of Escherichia coli: Gln-324 and Asn-325 are essential, and Ile-329-Val-339 form an alpha-helix.Mutant cycles at CFTR's non-canonical ATP-binding site support little interface separation during gatingRational Coupled Dynamics Network Manipulation Rescues Disease-Relevant Mutant Cystic Fibrosis Transmembrane Conductance Regulator.Cysteine accessibility probes timing and extent of NBD separation along the dimer interface in gating CFTR channels.Full-open and closed CFTR channels, with lateral tunnels from the cytoplasm and an alternative position of the F508 region, as revealed by molecular dynamics.A stable human-cell system overexpressing cystic fibrosis transmembrane conductance regulator recombinant protein at the cell surfaceCorrection of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809.Some gating potentiators, including VX-770, diminish ΔF508-CFTR functional expression.Thermal instability of ΔF508 cystic fibrosis transmembrane conductance regulator (CFTR) channel function: protection by single suppressor mutations and inhibiting channel activityDeletion of Phenylalanine 508 in the First Nucleotide-binding Domain of the Cystic Fibrosis Transmembrane Conductance Regulator Increases Conformational Exchange and Inhibits Dimerization.Phenylalanine-508 mediates a cytoplasmic-membrane domain contact in the CFTR 3D structure crucial to assembly and channel functionReview. ATP hydrolysis-driven gating in cystic fibrosis transmembrane conductance regulator.Interaction between 2 extracellular loops influences the activity of the cystic fibrosis transmembrane conductance regulator chloride channel.Functional stability of rescued delta F508 cystic fibrosis transmembrane conductance regulator in airway epithelial cells.Role of the juxtamembrane region of cytoplasmic loop 3 in the gating and conductance of the cystic fibrosis transmembrane conductance regulator chloride channel.Allosteric modulation balances thermodynamic stability and restores function of ΔF508 CFTR.Changes in accessibility of cytoplasmic substances to the pore associated with activation of the cystic fibrosis transmembrane conductance regulator chloride channel.Regulatory insertion removal restores maturation, stability and function of DeltaF508 CFTRCysteine-independent inhibition of the CFTR chloride channel by the cysteine-reactive reagent sodium (2-sulphonatoethyl) methanethiosulphonate.Multiple membrane-cytoplasmic domain contacts in the cystic fibrosis transmembrane conductance regulator (CFTR) mediate regulation of channel gating.Using a cysteine-less mutant to provide insight into the structure and mechanism of CFTR.NMR evidence for differential phosphorylation-dependent interactions in WT and DeltaF508 CFTR.Relationship between nucleotide binding and ion channel gating in cystic fibrosis transmembrane conductance regulator.A common mechanism for CFTR potentiators.Functional differences in pore properties between wild-type and cysteine-less forms of the CFTR chloride channel.Functional arrangement of the 12th transmembrane region in the CFTR chloride channel pore based on functional investigation of a cysteine-less CFTR variant.Molecular dynamics simulation study on the structural instability of the most common cystic fibrosis-associated mutant ΔF508-CFTR.
P2860
Q28533617-F5414609-1C84-4279-9319-8245E70AC2ADQ28658805-D706FCE4-8989-4F9E-9535-4B22218E29FCQ33283772-ECA4C9D7-FF26-4003-8878-D0A4D6114F9CQ34017049-01201AA8-AA63-48AB-B283-4F8C6C580EFFQ34096449-28CEA6A3-A4CC-4F4B-9A88-D9E4B5236090Q34578151-39FD9352-D35D-4554-8E3A-BCE43369F221Q35018175-0A4C7D86-26BB-46CE-8C59-327C088179BFQ35073390-6A4BF93E-3009-4903-A931-5A69C137CB54Q35234571-4180EF60-1F7C-4758-B087-EFE537AEE132Q35306053-2B7E68BA-0F71-4734-9220-2D6E48597F9AQ35514729-83B89B86-46F4-4316-8118-E98AC785428EQ35558817-068F776D-E969-4DD2-A88E-8742C26DABBAQ35743348-6B11596D-C7FD-4D2E-BB56-F8C335573809Q36108986-169FFABC-5DD2-4338-BE15-F27FF16C2683Q36281743-7BB657E5-F841-40EA-A0DD-15BF882A43A8Q36491305-69625C93-B1AC-4883-AC9C-C1AE47C62878Q37310608-C2D87F05-E8C6-4511-8443-75216B0D558DQ38953529-E1F5F625-B912-4D2B-BE9A-E56BF3DD6CE6Q39342530-4BE1AC38-3AB9-47AA-870E-C754AE10D368Q39356403-2E4A6438-CB10-4F78-BF26-2C453802A782Q39381996-806B962C-859D-4693-B069-797DE27479FDQ39673058-ED269232-970A-4099-BC54-051D95979629Q39691014-8E7F3879-68D1-4F5F-ADCF-9D364CD89ACAQ39847523-298AF7C7-4C03-4D3F-A158-694A0D4C547CQ39956881-F2080B06-7CBE-4306-A959-944C67114B08Q40312181-6638BF17-09AB-4133-98DB-316724088141Q41882930-D6122606-6F7C-4B3D-BA1D-F18A87654679Q47916154-A35C9340-7CA0-4D35-B908-E5857DD83C49Q48111581-E675746B-15A6-45EC-B68E-54399740976CQ51766345-E16330BB-17DE-480F-91C7-32F1FC4A47D1Q51766349-222E53FD-0FF2-4C97-9195-E32A494F5964Q55441392-F83F4E26-66C0-4C8B-86B8-6A879D61E33A
P2860
The role of cystic fibrosis transmembrane conductance regulator phenylalanine 508 side chain in ion channel gating.
description
2006 nî lūn-bûn
@nan
2006年の論文
@ja
2006年論文
@yue
2006年論文
@zh-hant
2006年論文
@zh-hk
2006年論文
@zh-mo
2006年論文
@zh-tw
2006年论文
@wuu
2006年论文
@zh
2006年论文
@zh-cn
name
The role of cystic fibrosis tr ...... e chain in ion channel gating.
@en
type
label
The role of cystic fibrosis tr ...... e chain in ion channel gating.
@en
prefLabel
The role of cystic fibrosis tr ...... e chain in ion channel gating.
@en
P2093
P2860
P1476
The role of cystic fibrosis tr ...... e chain in ion channel gating.
@en
P2093
Andrei A Aleksandrov
Jey-Hsin Chen
John R Riordan
Liying Cui
Luba Aleksandrov
Martina Gentzsch
Yue-Xian Hou
P2860
P304
P356
10.1113/JPHYSIOL.2005.099457
P407
P577
2006-02-16T00:00:00Z