Cargando…

Proteostasis Regulators in Cystic Fibrosis: Current Development and Future Perspectives

[Image: see text] In cystic fibrosis (CF), the deletion of phenylalanine 508 (F508del) in the CF transmembrane conductance regulator (CFTR) leads to misfolding and premature degradation of the mutant protein. These defects can be targeted with pharmacological agents named potentiators and correctors...

Descripción completa

Detalles Bibliográficos
Autores principales:	Brusa, Irene, Sondo, Elvira, Falchi, Federico, Pedemonte, Nicoletta, Roberti, Marinella, Cavalli, Andrea
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	American Chemical Society 2022
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9014417/ https://www.ncbi.nlm.nih.gov/pubmed/35377645 http://dx.doi.org/10.1021/acs.jmedchem.1c01897

Ejemplares similares

Innovative Strategy toward Mutant CFTR Rescue in Cystic Fibrosis: Design and Synthesis of Thiadiazole Inhibitors of the E3 Ligase RNF5
por: Brusa, Irene, et al.
Publicado: (2023)

Dual Activity of Aminoarylthiazoles on the Trafficking and Gating Defects of the Cystic Fibrosis Transmembrane Conductance Regulator Chloride Channel Caused by Cystic Fibrosis Mutations
por: Pedemonte, Nicoletta, et al.
Publicado: (2011)

Editorial: Special Issue on “Therapeutic Approaches for Cystic Fibrosis”
por: Pedemonte, Nicoletta
Publicado: (2020)

Proteomics and Metabolomics for Cystic Fibrosis Research
por: Liessi, Nara, et al.
Publicado: (2020)

Editorial: Emerging Therapeutic Approaches for Cystic Fibrosis
por: Lopes-Pacheco, Miquéias, et al.
Publicado: (2019)

Cystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis: current perspectives
por: Schmidt, Béla Z, et al.
Publicado: (2016)

Genetic Inhibition Of The Ubiquitin Ligase Rnf5 Attenuates Phenotypes Associated To F508del Cystic Fibrosis Mutation
por: Tomati, Valeria, et al.
Publicado: (2015)

Adapting Proteostasis and Autophagy for Controlling the Pathogenesis of Cystic Fibrosis Lung Disease
por: Bodas, Manish, et al.
Publicado: (2019)

High-Content Screening Identifies Vanilloids as a Novel Class of Inhibitors of NET Formation
por: Sondo, Elvira, et al.
Publicado: (2019)

Pharmaceutical Chaperones and Proteostasis Regulators in the Therapy of Lysosomal Storage Disorders: Current Perspective and Future Promises
por: Mohamed, Fedah E., et al.
Publicado: (2017)

Manipulating proteostasis to repair the F508del-CFTR defect in cystic fibrosis
por: Esposito, Speranza, et al.
Publicado: (2016)

The L467F-F508del Complex Allele Hampers Pharmacological Rescue of Mutant CFTR by Elexacaftor/Tezacaftor/Ivacaftor in Cystic Fibrosis Patients: The Value of the Ex Vivo Nasal Epithelial Model to Address Non-Responders to CFTR-Modulating Drugs
por: Sondo, Elvira, et al.
Publicado: (2022)

Chemically modified hCFTR mRNAs recuperate lung function in a mouse model of cystic fibrosis
por: Haque, A. K. M. Ashiqul, et al.
Publicado: (2018)

Cystic fibrosis: current therapeutic targets and future approaches
por: Rafeeq, Misbahuddin M., et al.
Publicado: (2017)

Distinctive lipid signatures of bronchial epithelial cells associated with cystic fibrosis drugs, including Trikafta
por: Liessi, Nara, et al.
Publicado: (2020)

Distinct proteostasis states drive pharmacologic chaperone susceptibility for cystic fibrosis transmembrane conductance regulator misfolding mutants
por: McDonald, Eli Fritz, et al.
Publicado: (2022)

Non-cystic fibrosis bronchiectasis in the elderly: current perspectives
por: Quinn, Tom M, et al.
Publicado: (2018)

Targeting the E1 ubiquitin-activating enzyme (UBA1) improves elexacaftor/tezacaftor/ivacaftor efficacy towards F508del and rare misfolded CFTR mutants
por: Borgo, Christian, et al.
Publicado: (2022)

Proteostasis and Mitochondrial Role on Psychiatric and Neurodegenerative Disorders: Current Perspectives
por: Olivero, Pablo, et al.
Publicado: (2018)

GM1 as Adjuvant of Innovative Therapies for Cystic Fibrosis Disease
por: Mancini, Giulia, et al.
Publicado: (2020)

Transcriptomic and Proteostasis Networks of CFTR and the Development of Small Molecule Modulators for the Treatment of Cystic Fibrosis Lung Disease
por: Strub, Matthew D., et al.
Publicado: (2020)

Mucosal humoral immunity in cystic fibrosis - a tangled web of failed proteostasis, infection and adaptive immunity
por: Moss, Richard B
Publicado: (2020)

Current landscape and future perspective of sentinel node mapping in endometrial cancer
por: Bogani, Giorgio, et al.
Publicado: (2018)

Sphingolipids and plasma membrane hydrolases in human primary bronchial cells during differentiation and their altered patterns in cystic fibrosis
por: Loberto, Nicoletta, et al.
Publicado: (2020)

Future therapies for cystic fibrosis
por: Allen, Lucy, et al.
Publicado: (2023)

Biological and Structural Basis for Aha1 Regulation of Hsp90 ATPase Activity in Maintaining Proteostasis in the Human Disease Cystic Fibrosis
por: Koulov, Atanas V., et al.
Publicado: (2010)

Cystic Fibrosis: Recent Insights into Inhaled Antibiotic Treatment and Future Perspectives
por: Taccetti, Giovanni, et al.
Publicado: (2021)

Proteostasis Takes Center Stage in Pulmonary Fibrosis
por: Budinger, G. R. Scott
Publicado: (2019)

Current Practices and Potential Nanotechnology Perspectives for Pain Related to Cystic Fibrosis
por: Trandafir, Laura M., et al.
Publicado: (2019)

Cystic Fibrosis: A New Target for 4-Imidazo[2,1-b]thiazole-1,4-dihydropyridines
por: Budriesi, Roberta, et al.
Publicado: (2011)

Acute Intermittent Porphyria: An Overview of Therapy Developments and Future Perspectives Focusing on Stabilisation of HMBS and Proteostasis Regulators
por: Bustad, Helene J., et al.
Publicado: (2021)

Therapeutic Approaches Targeting Proteostasis in Kidney Disease and Fibrosis
por: Chen, Jia-Huang, et al.
Publicado: (2021)

Targeting of Ubiquitin E3 Ligase RNF5 as a Novel Therapeutic Strategy in Neuroectodermal Tumors
por: Principi, Elisa, et al.
Publicado: (2022)

Simultaneous Quantification of Ivacaftor, Tezacaftor, and Elexacaftor in Cystic Fibrosis Patients’ Plasma by a Novel LC–MS/MS Method
por: Pigliasco, Federica, et al.
Publicado: (2023)

Preclinical testing of antimicrobials for cystic fibrosis lung infections: current needs and future priorities
por: Hubert, Lucile, et al.
Publicado: (2023)

CFTR Rescue by Lumacaftor (VX-809) Induces an Extensive Reorganization of Mitochondria in the Cystic Fibrosis Bronchial Epithelium
por: Braccia, Clarissa, et al.
Publicado: (2022)

Imaging in non-cystic fibrosis bronchiectasis and current limitations
por: Ledda, Roberta Eufrasia, et al.
Publicado: (2021)

A new future for patients with cystic fibrosis
por: Pullen, Lara C.
Publicado: (2020)

Pregnancy in Cystic Fibrosis—Past, Present, and Future
por: Gur, Michal, et al.
Publicado: (2023)

Future Comorbidities in an Aging Cystic Fibrosis Population
por: Ticona, Javier Humberto, et al.
Publicado: (2023)

Cannot write session to /tmp/vufind_sessions/sess_unju9q1dbuvo4cj12obeupqbeg