Cargando…

CFTR targeted therapies: recent advances in cystic fibrosis and possibilities in other diseases of the airways

Cystic fibrosis transmembrane conductance regulator (CFTR) is an ion transporter that regulates mucus hydration, viscosity and acidity of the airway epithelial surface. Genetic defects in CFTR impair regulation of mucus homeostasis, causing severe defects of mucociliary clearance as seen in cystic f...

Descripción completa

Detalles Bibliográficos
Autores principales:	Patel, Sheylan D., Bono, Taylor R., Rowe, Steven M., Solomon, George M.
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	European Respiratory Society 2020
Materias:	Series
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9131734/ https://www.ncbi.nlm.nih.gov/pubmed/32554756 http://dx.doi.org/10.1183/16000617.0068-2019

Ejemplares similares

The Effect of CFTR Modulators on Airway Infection in Cystic Fibrosis
por: Harvey, Caitlyn, et al.
Publicado: (2022)

Changes in the Cystic Fibrosis Airway Microbiome in Response to CFTR Modulator Therapy
por: Yi, Buqing, et al.
Publicado: (2021)

CFTR Modulator Therapies: Potential Impact on Airway Infections in Cystic Fibrosis
por: Saluzzo, Francesca, et al.
Publicado: (2022)

Recent Progress in CFTR Interactome Mapping and Its Importance for Cystic Fibrosis
por: Lim, Sang Hyun, et al.
Publicado: (2018)

Antisense oligonucleotide eluforsen improves CFTR function in F508del cystic fibrosis
por: Sermet-Gaudelus, Isabelle, et al.
Publicado: (2018)

Cystic Fibrosis, CFTR, and Colorectal Cancer
por: Scott, Patricia, et al.
Publicado: (2020)

Effects of CFTR modulators on serum biomarkers of liver fibrosis in children with cystic fibrosis
por: Levitte, Steven, et al.
Publicado: (2023)

CFTR and Ca(2+) Signaling in Cystic Fibrosis
por: Antigny, Fabrice, et al.
Publicado: (2011)

Strategies to circumvent the CFTR defect in cystic fibrosis
por: Becq, Frédéric, et al.
Publicado: (2013)

Not All Is CFTR – Neutrophils and Cholesterol in Cystic Fibrosis
por: Castellani, Stefano, et al.
Publicado: (2017)

Cystic Fibrosis and Oxidative Stress: The Role of CFTR
por: Moliteo, Evelina, et al.
Publicado: (2022)

CFTR modulators: transformative therapies for cystic fibrosis
por: Dwight, Mary, et al.
Publicado: (2021)

CFTR Modulators to the Rescue of Individuals with Cystic Fibrosis and Advanced Lung Disease
por: Myerburg, Mike, et al.
Publicado: (2021)

Ventilatory efficiency and its clinical and prognostic value in adults with cystic fibrosis
por: Laveneziana, Pierantonio, et al.
Publicado: (2021)

Corneal Refractive Surgery Considerations in Patients with Cystic Fibrosis and Cystic Fibrosis Transmembrane Conductance Regulator-Related Disorders
por: Moshirfar, Majid, et al.
Publicado: (2022)

The effect of CFTR modulators on a cystic fibrosis patient presenting with recurrent pancreatitis in the absence of respiratory symptoms: a case report
por: Johns, J. Dixon, et al.
Publicado: (2019)

CFTR Modulators: Impact on Fertility, Pregnancy, and Lactation in Women with Cystic Fibrosis
por: Taylor-Cousar, Jennifer L.
Publicado: (2020)

Cystic fibrosis modulator therapy can reverse cystic bronchiectasis
por: Middleton, Peter G., et al.
Publicado: (2023)

Expression of gain-of-function CFTR in cystic fibrosis airway cells restores epithelial function better than wild-type or codon-optimized CFTR
por: Woodall, Maximillian, et al.
Publicado: (2023)

A Novel G542X CFTR Rat Model of Cystic Fibrosis Is Sensitive to Nonsense Mediated Decay
por: Sharma, Jyoti, et al.
Publicado: (2020)

Advancing clinical development pathways for new CFTR modulators in cystic fibrosis
por: Mayer-Hamblett, Nicole, et al.
Publicado: (2016)

Inflammation and CFTR: might neutrophils be the key in cystic fibrosis?
por: Witko-Sarsat, V, et al.
Publicado: (1999)

Fixing cystic fibrosis by correcting CFTR domain assembly
por: Okiyoneda, Tsukasa, et al.
Publicado: (2012)

Targeted therapies to improve CFTR function in cystic fibrosis
por: Brodlie, Malcolm, et al.
Publicado: (2015)

Lived experiences of individuals with cystic fibrosis on CFTR-modulators
por: Page, Annelise, et al.
Publicado: (2022)

Nutritional impact of CFTR modulators in children with cystic fibrosis
por: Gaschignard, Margaux, et al.
Publicado: (2023)

Cross‐talk between CFTR and sphingolipids in cystic fibrosis
por: Dobi, Dorina, et al.
Publicado: (2023)

Cystic fibrosis gene modifier SLC26A9 modulates airway response to CFTR-directed therapeutics
por: Strug, Lisa J., et al.
Publicado: (2016)

The effect of CFTR modulators on structural lung disease in cystic fibrosis
por: Mok, L. Clara, et al.
Publicado: (2023)

Restoration of CFTR function in patients with cystic fibrosis carrying the F508del-CFTR mutation
por: Stefano, Daniela De, et al.
Publicado: (2014)

Airway microbial diversity is decreased in young children with cystic fibrosis compared to healthy controls but improved with CFTR modulation
por: Hahn, Andrea, et al.
Publicado: (2020)

Clinical development of triple-combination CFTR modulators for cystic fibrosis patients with one or two F508del alleles
por: Taylor-Cousar, Jennifer L., et al.
Publicado: (2019)

Assessment of p.Phe508del-CFTR functional restoration in pediatric primary cystic fibrosis airway epithelial cells
por: Sutanto, Erika N., et al.
Publicado: (2018)

Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Ubiquitylation as a Novel Pharmaceutical Target for Cystic Fibrosis
por: Fukuda, Ryosuke, et al.
Publicado: (2020)

Molecular Chaperones as Targets to Circumvent the CFTR Defect in Cystic Fibrosis
por: Chanoux, Rebecca A., et al.
Publicado: (2012)

Predicting CFTR activity with front-runner cystic fibrosis drugs
por: Becq, Frédéric
Publicado: (2015)

The importance of CFTR expression for neutrophil function in patients with Cystic Fibrosis
por: Jundi, B, et al.
Publicado: (2015)

The cystic fibrosis transmembrane conductance regulator (CFTR) and its stability
por: Meng, Xin, et al.
Publicado: (2016)

CFTR Modulators: Shedding Light on Precision Medicine for Cystic Fibrosis
por: Lopes-Pacheco, Miquéias
Publicado: (2016)

Triplet CFTR modulators: future prospects for treatment of cystic fibrosis
por: Chaudary, Nauman
Publicado: (2018)

Cannot write session to /tmp/vufind_sessions/sess_ns3ed4theuo27lim72l2kqv5qh