Conformational and temperature-sensitive stability defects of the delta F508 cystic fibrosis transmembrane conductance regulator in post-endoplasmic reticulum compartments.
about
Misfolding diverts CFTR from recycling to degradation: quality control at early endosomesProtein homeostasis at the plasma membraneCFTR Modulators: Shedding Light on Precision Medicine for Cystic FibrosisDiminished self-chaperoning activity of the DeltaF508 mutant of CFTR results in protein misfoldingPseudomonas aeruginosa inhibits endocytic recycling of CFTR in polarized human airway epithelial cellsSphingosine-1-Phosphate Is a Novel Regulator of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) ActivityRibosomal Stalk Protein Silencing Partially Corrects the ΔF508-CFTR Functional Expression DefectThe silent codon change I507-ATC->ATT contributes to the severity of the ΔF508 CFTR channel dysfunctionLMTK2-mediated phosphorylation regulates CFTR endocytosis in human airway epithelial cells.Pyrazolylthiazole as DeltaF508-cystic fibrosis transmembrane conductance regulator correctors with improved hydrophilicity compared to bithiazoles.Na+/H+ exchanger regulatory factor 1 overexpression-dependent increase of cytoskeleton organization is fundamental in the rescue of F508del cystic fibrosis transmembrane conductance regulator in human airway CFBE41o- cellsInterplay between ER exit code and domain conformation in CFTR misprocessing and rescue.Alteration of protein function by a silent polymorphism linked to tRNA abundance.Modulation of endocytic trafficking and apical stability of CFTR in primary human airway epithelial culturesConditional JAG1 mutation shows the developing heart is more sensitive than developing liver to JAG1 dosageSmall-molecule correctors of defective DeltaF508-CFTR cellular processing identified by high-throughput screening.Organic solutes rescue the functional defect in delta F508 cystic fibrosis transmembrane conductance regulator.Targets for cystic fibrosis therapy: proteomic analysis and correction of mutant cystic fibrosis transmembrane conductance regulatorCystic fibrosis transmembrane conductance regulator (CFTR) potentiators protect G551D but not ΔF508 CFTR from thermal instability.The cystic fibrosis-causing mutation deltaF508 affects multiple steps in cystic fibrosis transmembrane conductance regulator biogenesis.A genomic signature approach to rescue ΔF508-cystic fibrosis transmembrane conductance regulator biosynthesis and function.Pharmacological chaperone-mediated in vivo folding and stabilization of the P23H-opsin mutant associated with autosomal dominant retinitis pigmentosaEndocytic trafficking routes of wild type and DeltaF508 cystic fibrosis transmembrane conductance regulatorQuality control for unfolded proteins at the plasma membrane.Potentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosis.Rescuing protein conformation: prospects for pharmacological therapy in cystic fibrosisProtein processing and inflammatory signaling in Cystic Fibrosis: challenges and therapeutic strategies.Activation of endoplasmic reticulum stress response during the development of ischemic heart disease.Revertant mutants G550E and 4RK rescue cystic fibrosis mutants in the first nucleotide-binding domain of CFTR by different mechanisms.Genetics of familial intrahepatic cholestasis syndromes.Emerging drug treatments for cystic fibrosis.Thermally unstable gating of the most common cystic fibrosis mutant channel (ΔF508): "rescue" by suppressor mutations in nucleotide binding domain 1 and by constitutive mutations in the cytosolic loops.Effect of rapamycin on the fate of P23H opsin associated with retinitis pigmentosa (an American Ophthalmological Society thesis).Expression of three topologically distinct membrane proteins elicits unique stress response pathways in the yeast Saccharomyces cerevisiaeGenetic Inhibition Of The Ubiquitin Ligase Rnf5 Attenuates Phenotypes Associated To F508del Cystic Fibrosis Mutation.Structure-activity relationships of cyanoquinolines with corrector-potentiator activity in ΔF508 cystic fibrosis transmembrane conductance regulator proteinEndocytic trafficking of CFTR in health and disease.Development and characterization of synthetic antibodies binding to the cystic fibrosis conductance regulator.Comparative processing and function of human and ferret cystic fibrosis transmembrane conductance regulator.Thermal instability of ΔF508 cystic fibrosis transmembrane conductance regulator (CFTR) channel function: protection by single suppressor mutations and inhibiting channel activity
P2860
Q24676764-9A72C01D-CAAC-43C9-869E-D3951AEEFB36Q27009009-A9C17E8E-C8BC-43C0-B361-F9D9504A9ED3Q28073996-6E52408A-1F14-4EC1-A41C-CAB22913A56DQ28472340-7AC378ED-5668-4FC6-BDF0-63D6EA16B117Q28492469-495D8863-4147-4D9C-A4D1-E48B91C441A3Q28548390-6AF15EDC-D770-4E20-BB07-875CA12352EAQ28551966-25D2032D-A050-452F-AD7E-5B9D958A706DQ28658805-AA206A61-12FD-4AC2-87F8-2DA1082590CCQ30412916-B39C77A8-CD42-42C8-8FCC-2084450B4ED0Q33550492-9F7679F2-8C19-47E9-99FD-BE8460BAD404Q33571668-8FF9F3CB-FCA4-4FE7-934E-18AABF12D061Q33647033-81179998-A9D9-48A8-85B4-8BC8070136B9Q33693618-41464F39-438B-4499-8C4E-0EDEB0978405Q33728190-93DA27F1-D8C3-4662-8875-DB1CEA559EA0Q33905006-AD93C669-6FB9-4210-B757-250A2203D9EBQ33925348-2ED9C62C-59EF-422E-81BF-50AA05D5EBD4Q33973031-A37A88CD-89A7-4F5F-865D-512252B14982Q34082569-7811BDFB-648A-4D6D-A07E-796616B0B3C6Q34155612-89489121-EDE7-4373-B2CB-1FB85293E2E4Q34285307-2CDD7168-92E9-4902-893F-BF7C46990B37Q34305593-C3D6D931-8580-4A3C-B0F0-7FC0D19DD046Q34338708-82779A58-C59C-43B8-9681-0961F80D753FQ34359205-DBA95C88-8AAA-4BDA-9F79-C17828408691Q34412404-92F7894F-2E6D-4E01-935D-095D7EC241BAQ34748891-222D9FE1-BA18-4E23-BC8B-E11F5EABEAB2Q34817235-5450FC71-E1FA-457B-A005-DF3EC584744DQ35043552-ECAF59C9-00F1-4633-90CD-B815171CAE7DQ35047817-3CAF9B7D-664C-4BBD-A6B3-EEC31D1D5C30Q35215090-52105880-8284-46D3-B238-2805F83C9ACAQ35448570-79F00028-F73E-4E05-8E59-6C654BF12B53Q35602683-0A948272-B379-4F89-AA5C-D89F87D4AB35Q35605109-452F0CBA-EC40-4DD6-B116-0BCF2CA4BCFFQ35662681-911B66AC-0E11-42CF-9AF2-9A9C161486FFQ35671992-C5DA46BE-70C5-46D5-9840-5B84DE798A2CQ35693982-52A5CE65-1803-4695-910E-FF48F11B49CBQ35750147-935039DB-773B-4CEF-B14B-8C297C4278AEQ35971547-CCD96750-CCFF-4E6C-9A0E-ACF80EE0D579Q36019421-C9F0FA17-637C-412C-8C69-64955178EFFFQ36052358-17AA1CD9-1AEC-48A1-BC53-36182C3FBB5FQ36108986-5352BA20-617C-40B7-A15A-DD4C22894432
P2860
Conformational and temperature-sensitive stability defects of the delta F508 cystic fibrosis transmembrane conductance regulator in post-endoplasmic reticulum compartments.
description
2000 nî lūn-bûn
@nan
2000 թուականի Դեկտեմբերին հրատարակուած գիտական յօդուած
@hyw
2000 թվականի դեկտեմբերին հրատարակված գիտական հոդված
@hy
2000年の論文
@ja
2000年論文
@yue
2000年論文
@zh-hant
2000年論文
@zh-hk
2000年論文
@zh-mo
2000年論文
@zh-tw
2000年论文
@wuu
name
Conformational and temperature ...... lasmic reticulum compartments.
@ast
Conformational and temperature ...... lasmic reticulum compartments.
@en
type
label
Conformational and temperature ...... lasmic reticulum compartments.
@ast
Conformational and temperature ...... lasmic reticulum compartments.
@en
prefLabel
Conformational and temperature ...... lasmic reticulum compartments.
@ast
Conformational and temperature ...... lasmic reticulum compartments.
@en
P2093
P921
P356
P1476
Conformational and temperature ...... plasmic reticulum compartments
@en
P2093
P304
P356
10.1074/JBC.M009172200
P407
P577
2000-12-21T00:00:00Z