I. Pranke

发表

Emerging Therapeutic Approaches for Cystic Fibrosis. From Gene Editing to Personalized Medicine

I. Sermet-Gaudelus, A. Edelman, A. Hinzpeter, 2019, Front. Pharmacol..

Biosynthesis of cystic fibrosis transmembrane conductance regulator.

I. Sermet-Gaudelus, I. Pranke, 2014, The international journal of biochemistry & cell biology.

Factors influencing readthrough therapy for frequent cystic fibrosis premature termination codons

O. Namy, L. Bidou, Matthieu Coupet, 2018, ERJ Open Research.

Pharmacological chaperone-rescued cystic fibrosis CFTR-F508del mutant overcomes PRAF2-gated access to endoplasmic reticulum exit sites

S. Marullo, H. Enslen, I. Sermet-Gaudelus, 2022, Cellular and Molecular Life Sciences.

Differential CFTR-Interactome Proximity Labeling Procedures Identify Enrichment in Multiple SLC Transporters

S. Marullo, I. Guerrera, I. Sermet-Gaudelus, 2022, International journal of molecular sciences.

Pharmacological chaperone-rescued cystic fibrosis CFTR-F508del mutant overcomes PRAF2-gated access to endoplasmic reticulum exit sites

S. Marullo, H. Enslen, I. Sermet-Gaudelus, 2022, Cellular and Molecular Life Sciences.

Might Brushed Nasal Cells Be a Surrogate for CFTR Modulator Clinical Response?

I. Sermet-Gaudelus, A. Edelman, T. Flament, 2019, American journal of respiratory and critical care medicine.

Airway surface liquid acidification initiates host defense abnormalities in Cystic Fibrosis

L. Touqui, J. Sallenave, X. Nassif, 2019, Scientific Reports.

Keratin 8 is a scaffolding and regulatory protein of ERAD complexes

J. Irving, D. Lomas, G. Lukács, 2022, Cellular and Molecular Life Sciences.

α-Synuclein and ALPS motifs are membrane curvature sensors whose contrasting chemistry mediates selective vesicle binding

B. Antonny, C. L. Jackson, K. Gibson, 2011, The Journal of cell biology.

Promitotic Action of Oenothera biennis on Senescent Human Dermal Fibroblasts

S. Fumagalli, I. Guerrera, C. Cordier, 2022, International journal of molecular sciences.

Correction of CFTR function in nasal epithelial cells from cystic fibrosis patients predicts improvement of respiratory function by CFTR modulators

J. Jais, I. Sermet-Gaudelus, A. Edelman, 2017, Scientific Reports.

Acting on the CFTR Membrane-Spanning Domains Interface Rescues Some Misfolded Mutants

J. Mornon, I. Sermet-Gaudelus, I. Callebaut, 2022, International journal of molecular sciences.

Pharmacological chaperones improve intra-domain stability and inter-domain assembly via distinct binding sites to rescue misfolded CFTR

J. Mornon, I. Sermet-Gaudelus, I. Callebaut, 2021, Cellular and Molecular Life Sciences.

Cis variants identified in F508del complex alleles modulate CFTR channel rescue by small molecules

J. Mornon, I. Sermet-Gaudelus, I. Callebaut, 2018, Human mutation.

Modulators of CFTR. Updates on clinical development and future directions.

I. Sermet-Gaudelus, M. Semeraro, C. Gravier-Pelletier, 2021, European journal of medicinal chemistry.

Cystic Fibrosis Diagnosis in Newborns, Children, and Adults

C. Castellani, I. Sermet-Gaudelus, B. Linnane, 2019, Seminars in Respiratory and Critical Care Medicine.

The U UGA C sequence provides a favorable context to ELX-02 induced CFTR readthrough.

O. Namy, P. Reix, L. Bidou, 2022, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Pharmacological chaperones improve intra-domain stability and inter-domain assembly via distinct binding sites to rescue misfolded CFTR

J. Mornon, I. Sermet-Gaudelus, I. Callebaut, 2021, Cellular and Molecular Life Sciences.

Urinary Exosomes of Patients with Cystic Fibrosis Unravel CFTR-Related Renal Disease

L. Martignetti, I. Guerrera, I. Sermet-Gaudelus, 2020, International journal of molecular sciences.

Correlating genotype with phenotype using CFTR‐mediated whole‐cell Cl− currents in human nasal epithelial cells

I. Sermet-Gaudelus, N. Servel, D. Sheppard, 2021, The Journal of physiology.

The suppression of premature termination codons and the repair of splicing mutations in CFTR

B. Kerem, I. Sermet-Gaudelus, I. Pranke, 2017, Current opinion in pharmacology.

Isolation, cultivation, and application of primary respiratory epithelial cells obtained by nasal brushing, polyp samples, or lung explants

I. Sermet, D. Downey, L. Galietta, 2022, STAR Protocols.

CFTR corrector efficacy is associated with occupancy of distinct binding sites

J. Mornon, I. Sermet-Gaudelus, I. Callebaut, 2021, bioRxiv.

Sweat Chloride Testing and Nasal Potential Difference (NPD) Are Primary Outcome Parameters in Treatment with Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Modulators

I. Sermet-Gaudelus, T. Nguyen-Khoa, I. Pranke, 2021, Journal of personalized medicine.

Rattlesnake Phospholipase A2 Increases CFTR-Chloride Channel Current and Corrects ∆F508CFTR Dysfunction: Impact in Cystic Fibrosis.

P. Corringer, G. Lukács, F. Saul, 2016, Journal of molecular biology.

Systemic bis-phosphinic acid derivative restores chloride transport in Cystic Fibrosis mice

J. Jais, C. Schreiweis, S. Moriceau, 2022, Scientific Reports.

New insights into structure and function of bis-phosphinic acid derivatives and implications for CFTR modulation

J. Mornon, I. Sermet-Gaudelus, I. Callebaut, 2021, Scientific Reports.

An unexpected effect of TNF-α on F508del-CFTR maturation and function

I. Sermet-Gaudelus, N. Servel, A. Edelman, 2015, F1000Research.

Changes in lipid raft proteome upon TNF-α stimulation of cystic fibrosis cells.

M. Chanson, I. Guerrera, A. Edelman, 2016, Journal of proteomics.

Nonsense mutations accelerate lung disease and decrease survival of cystic fibrosis children.

B. Kerem, P. Reix, P. Burgel, 2023, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Rattlesnake Phospholipase A2 Increases CFTR-Chloride Channel Current and Corrects ∆F508CFTR Dysfunction: Impact in Cystic Fibrosis.

P. Corringer, G. Lukács, F. Saul, 2016, Journal of molecular biology.

Changes in lipid raft proteome upon TNF-α stimulation of cystic fibrosis cells.

I. Guerrera, C. Chhuon, I. Pranke, 2016, Journal of proteomics.

TLN468 changes the pattern of tRNA used to read through premature termination codons in CFTR

O. Namy, L. Bidou, I. Sermet-Gaudelus, 2024, bioRxiv.