Novel pharmacological strategies to treat cystic fibrosis.
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Pharmacological chaperoning: a primer on mechanism and pharmacologyRibosomal Stalk Protein Silencing Partially Corrects the ΔF508-CFTR Functional Expression DefectSynergy-based small-molecule screen using a human lung epithelial cell line yields ΔF508-CFTR correctors that augment VX-809 maximal efficacy.Using pharmacological chaperones to restore proteostasisA survey of detergents for the purification of stable, active human cystic fibrosis transmembrane conductance regulator (CFTR).Cystic fibrosis transmembrane conductance regulator chloride channel blockers: Pharmacological, biophysical and physiological relevance.Targeted proteomic quantitation of the absolute expression and turnover of cystic fibrosis transmembrane conductance regulator in the apical plasma membrane.Potentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosis.Targeting protein-protein interactions to rescue Δf508-cftr: a novel corrector approach to treat cystic fibrosis.Structurally distinct ligands rescue biogenesis defects of the KATP channel complex via a converging mechanism.A stable human-cell system overexpressing cystic fibrosis transmembrane conductance regulator recombinant protein at the cell surfaceDrug-set enrichment analysis: a novel tool to investigate drug mode of actionPotentiators of Defective ΔF508-CFTR Gating that Do Not Interfere with Corrector Action.Endoplasmic Reticulum-Targeted Subunit Toxins Provide a New Approach to Rescue Misfolded Mutant Proteins and Revert Cell Models of Genetic Diseases.Restoration of NBD1 thermal stability is necessary and sufficient to correct ∆F508 CFTR folding and assemblyModulating Innate and Adaptive Immunity by (R)-Roscovitine: Potential Therapeutic Opportunity in Cystic Fibrosis.Mechanism-based corrector combination restores ΔF508-CFTR folding and function.Pharmacological rescue of trafficking-impaired ATP-sensitive potassium channels.Mechanistic Insight into NMDA Receptor Dysregulation by Rare Variants in the GluN2A and GluN2B Agonist Binding Domains.Functional architecture of the CFTR chloride channel.A reciprocating twin-channel model for ABC transporters.Low free drug concentration prevents inhibition of F508del CFTR functional expression by the potentiator VX-770 (ivacaftor).Finding new drugs to enhance anion secretion in cystic fibrosis: Toward suitable systems for better drug screening. Report on the pre-conference meeting to the 12th ECFS Basic Science Conference, Albufeira, 25-28 March 2015.Clinical approach to the diagnosis and treatment of cystic fibrosis and CFTR-related disorders.CFTR potentiators partially restore channel function to A561E-CFTR, a cystic fibrosis mutant with a similar mechanism of dysfunction as F508del-CFTRTrimethylangelicin promotes the functional rescue of mutant F508del CFTR protein in cystic fibrosis airway cells.Regulation of Transporters and Channels by Membrane-Trafficking Complexes in Epithelial Cells.Conformational Stability and Pathogenic Misfolding of the Integral Membrane Protein PMP22.Armet/Manf and Creld2 are components of a specialized ER stress response provoked by inappropriate formation of disulphide bonds: implications for genetic skeletal diseases.Orphan diseases: state of the drug discovery art.Pharmacological Chaperones: Beyond Conformational Disorders.An Official American Thoracic Society Workshop Report: Translational Research in Rare Respiratory Diseases.Pharmacoperones as Novel Therapeutics for Diverse Protein Conformational Diseases.Structural mechanisms of CFTR function and dysfunction.Molecular Mechanism of Action of Trimethylangelicin Derivatives as CFTR Modulators.Identification and Characterization of Novel CFTR Potentiators
P2860
Q27001695-2013CBF1-5E84-49EB-9ECB-9FAB9B4E6DBAQ28551966-1F2A9989-733C-4D73-A5EC-0D34CBD84C49Q33742766-2D7EF010-80FF-403B-A19E-A83150CA7E91Q33804655-BC7D7027-D27A-4793-BAEA-82062AEB7340Q34221261-E83F8C2A-90DD-45AF-BB1D-B04DF6876A5FQ34408604-0083DB5F-FE35-43DF-8490-76D3A4E90D9CQ34488617-B987C7B4-D0D4-41BA-9C3A-0FB733ADA5F3Q34748891-D2E52AEB-46FE-422E-BECD-1288963ED76EQ34975372-7C6B7DEB-936D-4164-A126-6A9C57C8EA2AQ35199497-74EAC8E6-1B85-4E11-95C7-94E2CC571145Q35514729-744623DC-BB86-4EA1-9FD6-A3237E6FB5F3Q35790801-C2A1BE40-F154-44DF-BCDC-60D62389FCC5Q36077743-941B2761-DACD-414A-B94E-DCF0FDB9038CQ36217769-4AAD00F1-2112-463B-B95A-1EF69E8EFF63Q36591437-41CFC4E0-B96C-4BED-ABFD-35BE7B71B946Q36709312-8414E224-417B-4FB2-B026-1D08421EADDEQ37341854-F044D53A-7183-423F-816C-485653C4E3CCQ37409971-879AA565-3168-46B0-88D4-7ACD3FBF0D55Q37480684-2001A239-8212-46F8-B194-590462910499Q38171539-04A3E74B-54A5-48C6-AC08-E1B168A7A067Q38208521-DDAED4D2-FFCF-4CAB-B6CA-B0E49B3BF01FQ38825816-16712A96-E2C3-4D83-9C66-D19E8C34D7D2Q38910094-C01762F2-B86A-40C9-901D-721B176B9E1AQ38960863-EB04CB40-C26A-4319-8CA9-D618EE3179D1Q38987933-8944A508-C1A8-425C-90D7-A14980619BC3Q38996193-A152AD0F-705F-4F21-AC96-D54A5B9A3871Q39155176-851462AD-58F8-4D72-8455-E535D6E18273Q40808463-2523950E-2A69-4405-BD06-411C2C6ABBEBQ41964697-1DBCB62A-D21A-44F4-B9FB-AE4226E49619Q45882722-1BC64707-04E3-4F8E-BCDB-CDA0C6DD2E9DQ47602237-05E4E2FD-A83C-4C7E-9EEC-537136F9C46EQ47815265-770685ED-953D-468D-8379-6D0EC9734A70Q50014672-407F5DF2-9221-430C-BEFB-BF05F4F76A8DQ52629670-A989D7A9-62A0-4384-88A6-9FB551BAABFBQ55691070-F8A26A43-399E-402F-9DE8-025EB3389502Q58707750-D5144B05-2A7A-45DC-AF3D-4D16DB447ED5
P2860
Novel pharmacological strategies to treat cystic fibrosis.
description
article científic
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article scientifique
@fr
articol științific
@ro
articolo scientifico
@it
artigo científico
@gl
artigo científico
@pt
artigo científico
@pt-br
artikel ilmiah
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artikull shkencor
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artículo científico
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name
Novel pharmacological strategies to treat cystic fibrosis.
@en
type
label
Novel pharmacological strategies to treat cystic fibrosis.
@en
prefLabel
Novel pharmacological strategies to treat cystic fibrosis.
@en
P1476
Novel pharmacological strategies to treat cystic fibrosis
@en
P2093
Heidi M Sampson
John W Hanrahan
P304
P356
10.1016/J.TIPS.2012.11.006
P577
2013-02-01T00:00:00Z