These tools will no longer be maintained as of December 31, 2024. Archived website can be found here. PubMed4Hh GitHub repository can be found here. Contact NLM Customer Service if you have questions.


BIOMARKERS

Molecular Biopsy of Human Tumors

- a resource for Precision Medicine *

292 related articles for article (PubMed ID: 26976279)

  • 1. Manipulating proteostasis to repair the F508del-CFTR defect in cystic fibrosis.
    Esposito S; Tosco A; Villella VR; Raia V; Kroemer G; Maiuri L
    Mol Cell Pediatr; 2016 Dec; 3(1):13. PubMed ID: 26976279
    [TBL] [Abstract][Full Text] [Related]  

  • 2. Restoration of CFTR function in patients with cystic fibrosis carrying the F508del-CFTR mutation.
    De Stefano D; Villella VR; Esposito S; Tosco A; Sepe A; De Gregorio F; Salvadori L; Grassia R; Leone CA; De Rosa G; Maiuri MC; Pettoello-Mantovani M; Guido S; Bossi A; Zolin A; Venerando A; Pinna LA; Mehta A; Bona G; Kroemer G; Maiuri L; Raia V
    Autophagy; 2014; 10(11):2053-74. PubMed ID: 25350163
    [TBL] [Abstract][Full Text] [Related]  

  • 3. Targeting the Intracellular Environment in Cystic Fibrosis: Restoring Autophagy as a Novel Strategy to Circumvent the CFTR Defect.
    Villella VR; Esposito S; Bruscia EM; Maiuri MC; Raia V; Kroemer G; Maiuri L
    Front Pharmacol; 2013; 4():1. PubMed ID: 23346057
    [TBL] [Abstract][Full Text] [Related]  

  • 4. Efficacy of lumacaftor-ivacaftor for the treatment of cystic fibrosis patients homozygous for the F508del-CFTR mutation.
    Cholon DM; Esther CR; Gentzsch M
    Expert Rev Precis Med Drug Dev; 2016; 1(3):235-243. PubMed ID: 27482545
    [TBL] [Abstract][Full Text] [Related]  

  • 5. ORKAMBI-Mediated Rescue of Mucociliary Clearance in Cystic Fibrosis Primary Respiratory Cultures Is Enhanced by Arginine Uptake, Arginase Inhibition, and Promotion of Nitric Oxide Signaling to the Cystic Fibrosis Transmembrane Conductance Regulator Channel.
    Wu YS; Jiang J; Ahmadi S; Lew A; Laselva O; Xia S; Bartlett C; Ip W; Wellhauser L; Ouyang H; Gonska T; Moraes TJ; Bear CE
    Mol Pharmacol; 2019 Oct; 96(4):515-525. PubMed ID: 31427400
    [TBL] [Abstract][Full Text] [Related]  

  • 6. Cysteamine re-establishes the clearance of Pseudomonas aeruginosa by macrophages bearing the cystic fibrosis-relevant F508del-CFTR mutation.
    Ferrari E; Monzani R; Villella VR; Esposito S; Saluzzo F; Rossin F; D'Eletto M; Tosco A; De Gregorio F; Izzo V; Maiuri MC; Kroemer G; Raia V; Maiuri L
    Cell Death Dis; 2017 Jan; 8(1):e2544. PubMed ID: 28079883
    [TBL] [Abstract][Full Text] [Related]  

  • 7. Measurements of Functional Responses in Human Primary Lung Cells as a Basis for Personalized Therapy for Cystic Fibrosis.
    Awatade NT; Uliyakina I; Farinha CM; Clarke LA; Mendes K; Solé A; Pastor J; Ramos MM; Amaral MD
    EBioMedicine; 2015; 2(2):147-53. PubMed ID: 26137539
    [TBL] [Abstract][Full Text] [Related]  

  • 8. Efficacy and Safety of CFTR Corrector and Potentiator Combination Therapy in Patients with Cystic Fibrosis for the F508del-CFTR Homozygous Mutation: A Systematic Review and Meta-analysis.
    Wu HX; Zhu M; Xiong XF; Wei J; Zhuo KQ; Cheng DY
    Adv Ther; 2019 Feb; 36(2):451-461. PubMed ID: 30554331
    [TBL] [Abstract][Full Text] [Related]  

  • 9. Lumacaftor and ivacaftor in the management of patients with cystic fibrosis: current evidence and future prospects.
    Kuk K; Taylor-Cousar JL
    Ther Adv Respir Dis; 2015 Dec; 9(6):313-26. PubMed ID: 26416827
    [TBL] [Abstract][Full Text] [Related]  

  • 10. Unravelling the Regions of Mutant F508del-CFTR More Susceptible to the Action of Four Cystic Fibrosis Correctors.
    Amico G; Brandas C; Moran O; Baroni D
    Int J Mol Sci; 2019 Nov; 20(21):. PubMed ID: 31683989
    [TBL] [Abstract][Full Text] [Related]  

  • 11. Defective CFTR induces aggresome formation and lung inflammation in cystic fibrosis through ROS-mediated autophagy inhibition.
    Luciani A; Villella VR; Esposito S; Brunetti-Pierri N; Medina D; Settembre C; Gavina M; Pulze L; Giardino I; Pettoello-Mantovani M; D'Apolito M; Guido S; Masliah E; Spencer B; Quaratino S; Raia V; Ballabio A; Maiuri L
    Nat Cell Biol; 2010 Sep; 12(9):863-75. PubMed ID: 20711182
    [TBL] [Abstract][Full Text] [Related]  

  • 12. Effects of lumacaftor-ivacaftor therapy on cystic fibrosis transmembrane conductance regulator function in F508del homozygous patients with cystic fibrosis aged 2-11 years.
    Berges J; Graeber SY; Hämmerling S; Yu Y; Krümpelmann A; Stahl M; Hirtz S; Scheuermann H; Mall MA; Sommerburg O
    Front Pharmacol; 2023; 14():1188051. PubMed ID: 37324488
    [No Abstract]   [Full Text] [Related]  

  • 13. Unravelling druggable signalling networks that control F508del-CFTR proteostasis.
    Hegde RN; Parashuraman S; Iorio F; Ciciriello F; Capuani F; Carissimo A; Carrella D; Belcastro V; Subramanian A; Bounti L; Persico M; Carlile G; Galietta L; Thomas DY; Di Bernardo D; Luini A
    Elife; 2015 Dec; 4():. PubMed ID: 26701908
    [TBL] [Abstract][Full Text] [Related]  

  • 14. Pharmacological Correction of Cystic Fibrosis: Molecular Mechanisms at the Plasma Membrane to Augment Mutant CFTR Function.
    Arora K; Naren AP
    Curr Drug Targets; 2016; 17(11):1275-81. PubMed ID: 26648081
    [TBL] [Abstract][Full Text] [Related]  

  • 15. Adapting Proteostasis and Autophagy for Controlling the Pathogenesis of Cystic Fibrosis Lung Disease.
    Bodas M; Vij N
    Front Pharmacol; 2019; 10():20. PubMed ID: 30774592
    [TBL] [Abstract][Full Text] [Related]  

  • 16. Roscovitine is a proteostasis regulator that corrects the trafficking defect of F508del-CFTR by a CDK-independent mechanism.
    Norez C; Vandebrouck C; Bertrand J; Noel S; Durieu E; Oumata N; Galons H; Antigny F; Chatelier A; Bois P; Meijer L; Becq F
    Br J Pharmacol; 2014 Nov; 171(21):4831-49. PubMed ID: 25065395
    [TBL] [Abstract][Full Text] [Related]  

  • 17. Partial rescue of F508del-cystic fibrosis transmembrane conductance regulator channel gating with modest improvement of protein processing, but not stability, by a dual-acting small molecule.
    Liu J; Bihler H; Farinha CM; Awatade NT; Romão AM; Mercadante D; Cheng Y; Musisi I; Jantarajit W; Wang Y; Cai Z; Amaral MD; Mense M; Sheppard DN
    Br J Pharmacol; 2018 Apr; 175(7):1017-1038. PubMed ID: 29318594
    [TBL] [Abstract][Full Text] [Related]  

  • 18. Tezacaftor/Ivacaftor in Subjects with Cystic Fibrosis and F508del/F508del-CFTR or F508del/G551D-CFTR.
    Donaldson SH; Pilewski JM; Griese M; Cooke J; Viswanathan L; Tullis E; Davies JC; Lekstrom-Himes JA; Wang LT;
    Am J Respir Crit Care Med; 2018 Jan; 197(2):214-224. PubMed ID: 28930490
    [TBL] [Abstract][Full Text] [Related]  

  • 19. Airway Epithelial Inflammation
    Gentzsch M; Cholon DM; Quinney NL; Martino MEB; Minges JT; Boyles SE; Guhr Lee TN; Esther CR; Ribeiro CMP
    Front Pharmacol; 2021; 12():628722. PubMed ID: 33859562
    [TBL] [Abstract][Full Text] [Related]  

  • 20. Efficacy and safety of lumacaftor and ivacaftor in patients aged 6-11 years with cystic fibrosis homozygous for F508del-CFTR: a randomised, placebo-controlled phase 3 trial.
    Ratjen F; Hug C; Marigowda G; Tian S; Huang X; Stanojevic S; Milla CE; Robinson PD; Waltz D; Davies JC;
    Lancet Respir Med; 2017 Jul; 5(7):557-567. PubMed ID: 28606620
    [TBL] [Abstract][Full Text] [Related]  

    [Next]    [New Search]
    of 15.