Cargando…

Characterization of two rat models of cystic fibrosis—KO and F508del CFTR—Generated by Crispr‐Cas9

BACKGROUND: Genetically engineered animals are essential for gaining a proper understanding of the disease mechanisms of cystic fibrosis (CF). The rat is a relevant laboratory model for CF because of its zootechnical capacity, size, and airway characteristics, including the presence of submucosal gl...

Descripción completa

Detalles Bibliográficos
Autores principales:	Dreano, Elise, Bacchetta, Marc, Simonin, Juliette, Galmiche, Louise, Usal, Claire, Slimani, Lotfi, Sadoine, Jérémy, Tesson, Laurent, Anegon, Ignacio, Concordet, Jean‐Paul, Hatton, Aurélie, Vignaud, Lucile, Tondelier, Danielle, Sermet‐Gaudelus, Isabelle, Chanson, Marc, Cottart, Charles‐Henry
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	John Wiley and Sons Inc. 2019
Materias:	Original Articles
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6930998/ https://www.ncbi.nlm.nih.gov/pubmed/31942562 http://dx.doi.org/10.1002/ame2.12091

Ejemplares similares

An unexpected effect of TNF-α on F508del-CFTR maturation and function
por: Bitam, Sara, et al.
Publicado: (2015)

Characterization of Nasal Potential Difference in cftr Knockout and F508del-CFTR Mice
por: Saussereau, Emilie Lyne, et al.
Publicado: (2013)

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

Sweat Chloride Testing and Nasal Potential Difference (NPD) Are Primary Outcome Parameters in Treatment with Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Modulators
por: Sermet-Gaudelus, Isabelle, et al.
Publicado: (2021)

Production of CFTR-ΔF508 Rabbits
por: Yang, Dongshan, et al.
Publicado: (2021)

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

Discovery of novel potent ΔF508-CFTR correctors that target the nucleotide binding domain
por: Odolczyk, Norbert, et al.
Publicado: (2013)

Correction of CFTR function in nasal epithelial cells from cystic fibrosis patients predicts improvement of respiratory function by CFTR modulators
por: Pranke, Iwona M., et al.
Publicado: (2017)

Recovery of ΔF508-CFTR Function by Citrate
por: Borkenhagen, Beatrice, et al.
Publicado: (2022)

Defective Fluid Secretion from Submucosal Glands of Nasal Turbinates from CFTR(-/-) and CFTR(ΔF508/ΔF508) Pigs
por: Cho, Hyung-Ju, et al.
Publicado: (2011)

Extracellular phosphate enhances the function of F508del-CFTR rescued by CFTR correctors
por: Saint-Criq, Vinciane, et al.
Publicado: (2021)

New insights into structure and function of bis-phosphinic acid derivatives and implications for CFTR modulation
por: Bitam, Sara, et al.
Publicado: (2021)

Unsolved severe chronic rhinosinusitis elucidated by extensive CFTR genotyping
por: Degrugillier, Fanny, et al.
Publicado: (2019)

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

Benzbromarone Stabilizes ΔF508 CFTR at the Cell Surface
por: Loo, Tip W., et al.
Publicado: (2011)

In vivo model for modulators of CFTR(ΔF508) misfolding
Publicado: (2012)

Correctors modify the bicarbonate permeability of F508del-CFTR
por: Fiore, Michele, et al.
Publicado: (2020)

Airway Surface Liquid pH Regulation in Airway Epithelium Current Understandings and Gaps in Knowledge
por: Zajac, Miroslaw, et al.
Publicado: (2021)

Author Correction: New insights into structure and function of bis-phosphinic acid derivatives and implications for CFTR modulation
por: Bitam, Sara, et al.
Publicado: (2021)

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

CFTR-F508 pigs provide new clues to cystic fibrosis
Publicado: (2011)

Syntaxin 8 and the Endoplasmic Reticulum Processing of ΔF508-CFTR
por: Sabirzhanova, Inna, et al.
Publicado: (2018)

Structural Comparative Modeling of Multi-Domain F508del CFTR
por: McDonald, Eli Fritz, et al.
Publicado: (2022)

Airway surface liquid acidification initiates host defense abnormalities in Cystic Fibrosis
por: Simonin, Juliette, et al.
Publicado: (2019)

Author Correction: Airway surface liquid acidification initiates host defense abnormalities in Cystic Fibrosis
por: Simonin, Juliette, et al.
Publicado: (2019)

Functional Rescue of F508del-CFTR Using Small Molecule Correctors
por: Molinski, Steven, et al.
Publicado: (2012)

Unravelling druggable signalling networks that control F508del-CFTR proteostasis
por: Hegde, Ramanath Narayana, et al.
Publicado: (2015)

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

ΔF508 CFTR interactome remodeling promotes rescue of Cystic Fibrosis
por: Pankow, Sandra, et al.
Publicado: (2015)

Molecular Structures Reveal Synergistic Rescue of Δ508 CFTR by Trikafta Modulators
por: Fiedorczuk, Karol, et al.
Publicado: (2022)

The CFTR Amplifier Nesolicaftor Rescues TGF-β1 Inhibition of Modulator-Corrected F508del CFTR Function
por: Bengtson, Charles, et al.
Publicado: (2022)

Combined Bacteriophage and Antibiotic Treatment Prevents Pseudomonas aeruginosa Infection of Wild Type and cftr- Epithelial Cells
por: Luscher, Alexandre, et al.
Publicado: (2020)

Immunosuppressive role of fibrinogen-like protein 2 (FGL2) in CD8(+)regulatory T cells-mediated long-term graft survival
por: Bézie, Séverine, et al.
Publicado: (2011)

Full Rescue of F508del-CFTR Processing and Function by CFTR Modulators Can Be Achieved by Removal of Two Regulatory Regions
por: Uliyakina, Inna, et al.
Publicado: (2020)

Analysis of multiple gene co-expression networks to discover interactions favoring CFTR biogenesis and ΔF508-CFTR rescue
por: Strub, Matthew D., et al.
Publicado: (2021)

Is CFTR-delF508 Really Absent from the Apical Membrane of the Airway Epithelium?
por: Borthwick, Lee A., et al.
Publicado: (2011)

Biophysical Characterisation of Calumenin as a Charged F508del-CFTR Folding Modulator
por: Tripathi, Rashmi, et al.
Publicado: (2014)

Dissection of the Role of VIMP in Endoplasmic Reticulum-Associated Degradation of CFTRΔF508
por: Hou, Xia, et al.
Publicado: (2018)

CFTR:F508d/A613T Mutation Is Associated With Recurrent Episodes of Pancreatitis
por: Lin, Cindy, et al.
Publicado: (2021)

Acting on the CFTR Membrane-Spanning Domains Interface Rescues Some Misfolded Mutants
por: Baatallah, Nesrine, et al.
Publicado: (2022)

Cannot write session to /tmp/vufind_sessions/sess_m9ajlgblk27lqoknl98q9dab1h