I. Sermet-Gaudelus

发表

A CFTR potentiator in patients with cystic fibrosis and the G551D mutation.

Matthias Griese, P. Dřevínek, N. McElvaney, 2011, The New England journal of medicine.

In vitroprediction of stop-codon suppression by intravenous gentamicin in patients with cystic fibrosis: a pilot study

J. Rousset, L. Bidou, I. Sermet-Gaudelus, 2007 .

Myeloperoxidase Promoter Polymorphism −463G Is Associated With More Severe Clinical Expression of Cystic Fibrosis Pulmonary Disease

I. Sermet-Gaudelus, B. Descamps-Latscha, G. Lenoir, 2006, Mediators of inflammation.

ECFS best practice guidelines: the 2018 revision.

C. Castellani, P. Dřevínek, S. Bell, 2018, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

US Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus recommendations for the management of non-tuberculous mycobacteria in individuals with cystic fibrosis: executive summary

R. Gibson, L. Saiman, P. Corris, 2015, Thorax.

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

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

Ataluren (PTC124) Induces CFTR Protein Expression and Activity in Children with Nonsense Mutation Cystic Fibrosis

F. Vermeulen, I. Sermet-Gaudelus, T. Ajayi, 2010 .

An international randomized multicenter comparison of nasal potential difference techniques.

J. Clancy, F. Accurso, F. Vermeulen, 2010, Chest.

The very low penetrance of cystic fibrosis for the R117H mutation: a reappraisal for genetic counselling and newborn screening

E. Génin, C. Clavel, S. Leroy, 2009, Journal of Medical Genetics.

Chloride transport in nasal ciliated cells of cystic fibrosis heterozygotes.

I. Hurbain, I. Sermet-Gaudelus, J. Bresson, 2005, American journal of respiratory and critical care medicine.

Analysis of nasal potential in murine cystic fibrosis models.

J. Jais, I. Sermet-Gaudelus, A. Edelman, 2016, The international journal of biochemistry & cell biology.

Structure-guided combination therapy to potently improve the function of mutant CFTRs

A. Orth, P. McNamara, G. Lukács, 2018, Nature Medicine.

Biosynthesis of cystic fibrosis transmembrane conductance regulator.

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

Coronin-1 Is Associated with Neutrophil Survival and Is Cleaved during Apoptosis: Potential Implication in Neutrophils from Cystic Fibrosis Patients1

I. Guerrera, I. Sermet-Gaudelus, C. Danel, 2009, The Journal of Immunology.

Mycobacterium abscessus and Children with Cystic Fibrosis

P. Berche, I. Sermet-Gaudelus, J. Gaillard, 2003, Emerging infectious diseases.

Mycobacterium bolletii Lung Disease in Cystic Fibrosis.

J. Jais, I. Sermet-Gaudelus, J. Gaillard, 2019, Chest.

Measurement of nasal potential difference in young children with an equivocal sweat test following newborn screening for cystic fibrosis

C. Thauvin-Robinet, I. Sermet-Gaudelus, I. Fajac, 2010, Thorax.

Sequence PCR-Based DiversiLab System Repetitive Extragenic Palindromic as Determined Using the Semiautomated among Mycobacterium abscessus Isolates Clonal Relationship and Differentiation

X. Nassif, I. Sermet-Gaudelus, J. Gaillard, 2014 .

Clonal Relationship and Differentiation among Mycobacterium abscessus Isolates as Determined Using the Semiautomated Repetitive Extragenic Palindromic Sequence PCR-Based DiversiLab System

X. Nassif, I. Sermet-Gaudelus, J. Gaillard, 2014, Journal of Clinical Microbiology.

P. Berche, I. Sermet-Gaudelus, J. Gaillard, 2005, Journal of Clinical Microbiology.

Inflammation biomarkers in sputum for clinical trials in cystic fibrosis: current understanding and gaps in knowledge.

P. Burgel, L. Dupont, I. Sermet-Gaudelus, 2021, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

A critical review of definitions used to describe Pseudomonas aeruginosa microbiological status in patients with cystic fibrosis for application in clinical trials.

B. Tümmler, P. Dřevínek, E. Nash, 2019, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Ataluren for the treatment of nonsense-mutation cystic fibrosis: a randomised, double-blind, placebo-controlled phase 3 trial.

S. Peltz, S. McColley, E. Kerem, 2014, The Lancet. Respiratory medicine.

Ataluren (PTC124) induces cystic fibrosis transmembrane conductance regulator protein expression and activity in children with nonsense mutation cystic fibrosis.

F. Vermeulen, I. Sermet-Gaudelus, T. Ajayi, 2010, American journal of respiratory and critical care medicine.

Value of the Chlorhexidine Decontamination Method for Recovery of Nontuberculous Mycobacteria from Sputum Samples of Patients with Cystic Fibrosis

P. Berche, I. Sermet-Gaudelus, J. Gaillard, 2006, Journal of Clinical Microbiology.

[Weakening osteopathies, chronic kidney disease, malabsorption, biological anomalies of calium/phosphorus metabolism: appropriate indications for a reasoned reimbursment of serum vitamin D measurement].

P. Chanson, C. Annweiler, O. Beauchet, 2014, Annales de biologie clinique.

Elexacaftor/Tezacaftor/Ivacaftor alters branching morphogenesis of the mouse embryonic lung

C. Delacourt, Nicolas Houde, I. Sermet-Gaudelus, 2021, bioRxiv.

Bone demineralization is improved by ivacaftor in patients with cystic fibrosis carrying the p.Gly551Asp mutation.

I. Durieu, I. Sermet-Gaudelus, D. Hubert, 2016, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Increased expression of ATP12A proton pump in cystic fibrosis airways.

I. Sermet-Gaudelus, A. Palleschi, D. Tosi, 2018, JCI insight.

CFTR functional measurements in human models for diagnosis, prognosis and personalized therapy: Report on the pre-conference meeting to the 11th ECFS Basic Science Conference, Malta, 26-29 March 2014.

M. Drumm, A. Mehta, M. Amaral, 2014, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Interest of colchicine for the treatment of cystic fibrosis patients. Preliminary report.

I. Sermet-Gaudelus, B. Descamps-Latscha, P. Reinert, 1999, Mediators of Inflammation.

Cystic Fibrosis-Related Bone Disease: Current Knowledge and Future Directions

I. Durieu, I. Sermet-Gaudelus, S. Guérin, 2020 .

Cystic fibrosis bone disease treatment: Current knowledge and future directions.

I. Sermet-Gaudelus, M. Putman, V. Tangpricha, 2019, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Comparison of Transient Elastography, ShearWave Elastography, Magnetic Resonance Elastography and FibroTest as routine diagnostic markers for assessing liver fibrosis in children with Cystic Fibrosis.

I. Sermet-Gaudelus, Jérémy Dana, F. Imbert‐Bismut, 2021, Clinics and research in hepatology and gastroenterology.

Cystic Fibrosis Patients Potential Implication in Neutrophils from Survival and Is Cleaved during Apoptosis: Coronin-1 Is Associated with Neutrophil

I. Guerrera, I. Sermet-Gaudelus, C. Danel, 2009 .

Determinants of Refusal of A/H1N1 Pandemic Vaccination in a High Risk Population: A Qualitative Approach

I. Sermet-Gaudelus, O. Lortholary, O. Launay, 2012, PloS one.

AGER -429T/C Is Associated with an Increased Lung Disease Severity in Cystic Fibrosis

P. Böelle, P. Reix, J. Dalphin, 2012, PloS one.

Evaluation of MRP1-5 Gene Expression in Cystic Fibrosis Patients Homozygous for the ΔF508 Mutation

J. Bernaudin, I. Hurbain, I. Sermet-Gaudelus, 2003, Pediatric Research.

Insulin-like growth factor I correlates with lean body mass in cystic fibrosis patients

J. Souberbielle, I. Sermet-Gaudelus, G. Lenoir, 2003, Archives of disease in childhood.

Resolvin D1 regulates epithelial ion transport and inflammation in cystic fibrosis airways.

B. Harvey, I. Sermet-Gaudelus, V. Urbach, 2017, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

[Nontuberculous mycobacteria in cystic fibrosis].

I. Sermet-Gaudelus, J. Gaillard, É. Catherinot, 2005, Archives de pediatrie : organe officiel de la Societe francaise de pediatrie.

Reclassifying inconclusive diagnosis after newborn screening for cystic fibrosis. Moving forward.

M. Claustres, I. Sermet-Gaudelus, E. Girodon, 2021, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Guidelines for the clinical management and follow-up of infants with inconclusive cystic fibrosis diagnosis through newborn screening.

I. Sermet-Gaudelus, M. Audrézet, E. Girodon, 2017, Archives de pediatrie : organe officiel de la Societe francaise de pediatrie.

Diagnosis of Cystic Fibrosis in Screened Populations

D. Sanders, M. Rosenfeld, H. Blau, 2017, The Journal of pediatrics.

CFTR biomarkers: time for promotion to surrogate end-point

M. Amaral, N. Derichs, M. Proesmans, 2012, European Respiratory Journal.

Bioelectrical impedance in young patients with cystic fibrosis: Validation of a specific equation and clinical relevance.

J. Jais, I. Sermet-Gaudelus, G. Casimir, 2016, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Chronic Staphylococcus aureus lung infection correlates with proteogenomic and metabolic adaptations leading to an increased intracellular persistence

A. Charbit, I. Guerrera, X. Nassif, 2018, bioRxiv.

Update on cystic fibrosis-related bone disease: a special focus on children.

I. Sermet-Gaudelus, R. Aris, M. Castanet, 2009, Paediatric respiratory reviews.

[Bone health in cystic fibrosis].

J. Souberbielle, I. Sermet-Gaudelus, M. Castanet, 2009, Archives de pediatrie : organe officiel de la Societe francaise de pediatrie.

Low bone mineral density in young children with cystic fibrosis.

J. Souberbielle, I. Sermet-Gaudelus, G. Lenoir, 2007, American journal of respiratory and critical care medicine.

Cystic fibrosis and bone disease: defective osteoblast maturation with the F508del mutation in cystic fibrosis transmembrane conductance regulator.

L. Touqui, I. Sermet-Gaudelus, C. Guillaume, 2014, American journal of respiratory and critical care medicine.

Cystic fibrosis transmembrane conductance regulator (CFTR) regulates the production of osteoprotegerin (OPG) and prostaglandin (PG) E2 in human bone.

L. Touqui, I. Sermet-Gaudelus, C. Guillaume, 2010, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Efficacy and safety of ataluren in patients with nonsense-mutation cystic fibrosis not receiving chronic inhaled aminoglycosides: The international, randomized, double-blind, placebo-controlled Ataluren Confirmatory Trial in Cystic Fibrosis (ACT CF).

E. Kerem, M. Konstan, I. Sermet-Gaudelus, 2017, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Factors influencing readthrough therapy for frequent cystic fibrosis premature termination codons

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

Genetic diagnosis in practice: From cystic fibrosis to CFTR-related disorders.

P. Burgel, I. Sermet-Gaudelus, A. Pagin, 2020, Archives de pediatrie : organe officiel de la Societe francaise de pediatrie.

Predictive factors for lumacaftor/ivacaftor clinical response.

I. Sermet-Gaudelus, E. Girodon, A. Edelman, 2019, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Ivacaftor treatment in patients with cystic fibrosis and the G551D-CFTR mutation

I. Sermet-Gaudelus, 2013, European Respiratory Review.

Clinical response to lumacaftor-ivacaftor in patients with cystic fibrosis according to baseline lung function.

P. Reix, P. Burgel, J. Paillasseur, 2020, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

The hsa-miR-125a/hsa-let-7e/hsa-miR-99b cluster is potentially implicated in Cystic Fibrosis pathogenesis.

L. Mazzolini, I. Sermet-Gaudelus, T. Bienvenu, 2015, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Guidelines for the management of children at risk of secondary bone fragility: Expert opinion of a French working group.

J. Salles, J. Bacchetta, E. Dugelay, 2020, Archives de pediatrie : organe officiel de la Societe francaise de pediatrie.

A randomized placebo-controlled trial of ataluren for the treatment of nonsense mutation cystic fibrosis

S. Peltz, S. McColley, F. Accurso, 2014 .

Continuous versus Intermittent Infusions of Ceftazidime for Treating Exacerbation of Cystic Fibrosis (cid:1)

C. Cracowski, B. Wallaert, I. Sermet-Gaudelus, 2009 .

Genetic testing to provide targeted treatment for cystic fibrosis patients.

J. Rousset, I. Sermet-Gaudelus, A. Edelman, 2007, Pharmacogenomics.

A Phase 3, open-label, 96-week trial to study the safety, tolerability, and efficacy of tezacaftor/ivacaftor in children ≥ 6 years of age homozygous for F508del or heterozygous for F508del and a residual function CFTR variant.

G. Sawicki, C. Wainwright, I. Sermet-Gaudelus, 2022, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

[Recommendations for the management of bone demineralization in cystic fibrosis].

G. Dacremont, J. Souberbielle, I. Sermet-Gaudelus, 2008, Archives de pediatrie : organe officiel de la Societe francaise de pediatrie.

Pain in children and adults with cystic fibrosis: a comparative study.

I. Sermet-Gaudelus, G. Lenoir, D. Dusser, 2009, Journal of pain and symptom management.

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.

Association between Staphylococcus aureus alone or combined with Pseudomonas aeruginosa and the clinical condition of patients with cystic fibrosis.

P. Burgel, J. Coste, I. Sermet-Gaudelus, 2013, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

ECFS standards of care on CFTR-related disorders: Diagnostic criteria of CFTR dysfunction.

F. Vermeulen, I. Sermet-Gaudelus, P. Melotti, 2022, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Updated guidance on the management of children with cystic fibrosis transmembrane conductance regulator-related metabolic syndrome/cystic fibrosis screen positive, inconclusive diagnosis (CRMS/CFSPID).

O. Sommerburg, S. McColley, C. Castellani, 2020, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Nasal potential difference in cystic fibrosis diagnosis of very young children.

I. Sermet-Gaudelus, E. Girodon, G. Lenoir, 2007, The Journal of pediatrics.

The CF-CIRC study: a French collaborative study to assess the accuracy of Cystic Fibrosis diagnosis in neonatal screening

P. Reix, G. Bellon, I. Sermet-Gaudelus, 2006, BMC pediatrics.

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

M. Bacchetta, J. Concordet, M. Chanson, 2019, Animal models and experimental medicine.

Antisense oligonucleotide-based drug development for Cystic Fibrosis patients carrying the 3849 + 10 kb C-to-T splicing mutation

B. Kerem, L. Carmel, E. Kerem, 2021, bioRxiv.

Sputum Itraconazole Concentrations in Cystic Fibrosis Patients

P. Berche, I. Sermet-Gaudelus, C. Hennequin, 2001, Antimicrobial Agents and Chemotherapy.

Genotypic and Phenotypic Variation in Pseudomonas aeruginosa Reveals Signatures of Secondary Infection and Mutator Activity in Certain Cystic Fibrosis Patients with Chronic Lung Infections (cid:1)

F. Taddei, I. Sermet-Gaudelus, M. Franklin, 2011 .

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.

The global impact of SARS-CoV-2 in 181 people with cystic fibrosis

C. Goss, H. Rodgers, P. Burgel, 2020, Journal of Cystic Fibrosis.

Emerging medicines to improve the basic defect in cystic fibrosis

I. Sermet-Gaudelus, I. Fajac, 2022, Expert opinion on emerging drugs.

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.

"Il faut continuer à poser des questions" patient reported outcome measures in cystic fibrosis: An anthropological perspective.

P. Burgel, I. Sermet-Gaudelus, J. Bend, 2021, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Airway surface liquid acidification initiates host defense abnormalities in Cystic Fibrosis

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

Nebulized Antibiotics in Cystic Fibrosis

I. Sermet-Gaudelus, G. Lenoir, A. Ferroni, 2002, Paediatric drugs.

ECFS standards of care on CFTR-related disorders: Updated diagnostic criteria.

C. Castellani, I. Sermet-Gaudelus, K. De Boeck, 2022, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Keratin 8 is a scaffolding and regulatory protein of ERAD complexes

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

Neuroimaging manifestations in children with SARS-CoV-2 infection: a multinational, multicentre collaborative study

P. Grant, D. Evans, D. Seidenwurm, 2020, The Lancet Child & Adolescent Health.

Insights into the variability of nasal potential difference, a biomarker of CFTR activity.

J. Jais, J. Davies, I. Sermet-Gaudelus, 2019, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Saliva for molecular detection of SARS‐CoV‐2 in pre‐school and school‐age children

I. Sermet-Gaudelus, B. Bader-Meunier, P. Quartier, 2022, Environmental microbiology.

Chapter 10 Channelopathies in bronchiectasis

I. Sermet-Gaudelus, I. Fajac, A. Edelman, 2011 .

Real-Life Safety and Effectiveness of Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis.

D. Caimmi, A. Didier, S. Leroy, 2020, American journal of respiratory and critical care medicine.

- Molecular biology of cystic fibrosis: CFTR processing and functions and classes of mutations

I. Sermet-Gaudelus, P. Fanen, 2015 .

Use of 16S rRNA Gene Sequencing for Identification of Nonfermenting Gram-Negative Bacilli Recovered from Patients Attending a Single Cystic Fibrosis Center

P. Berche, E. Abachin, I. Sermet-Gaudelus, 2002, Journal of Clinical Microbiology.

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.

A multiple reader scoring system for Nasal Potential Difference parameters.

F. Vermeulen, I. Sermet-Gaudelus, S. Rowe, 2017, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Airway surface hyperviscosity and defective mucociliary transport by IL-17/TNF-α are corrected by β-adrenergic stimulus

S. Sarnataro, I. Sermet-Gaudelus, R. De Cegli, 2022, JCI insight.

Channelopathies in bronchiectasis

I. Sermet-Gaudelus, I. Fajac, A. Edelman, 2011 .

Clinical changes of patients with cystic fibrosis during transition from pediatric to adult care.

I. Sermet-Gaudelus, Isabelle Sermet-Gaudelus, M. Le Bourgeois, 2008, The Journal of adolescent health : official publication of the Society for Adolescent Medicine.

1-Serological methods: description of LIPS assay

D. Kariyawasam, C. Huon, I. Sermet-Gaudelus, 2021 .

A multinational report to characterise SARS-CoV-2 infection in people with cystic fibrosis

C. Goss, H. Rodgers, P. Burgel, 2020, Journal of Cystic Fibrosis.

Pseudomonas aeruginosa eradicates Staphylococcus aureus by manipulating the host immunity

D. Leduc, L. Touqui, P. Burgel, 2014, Nature Communications.

Matrix-Assisted Laser Desorption Ionization-Time of Flight Mass Spectrometry for Identification of Nonfermenting Gram-Negative Bacilli Isolated from Cystic Fibrosis Patients

P. Berche, X. Nassif, I. Sermet-Gaudelus, 2008, Journal of Clinical Microbiology.

Long-Term Rasamsonia argillacea Complex Species Colonization Revealed by PCR Amplification of Repetitive DNA Sequences in Cystic Fibrosis Patients

G. Gargala, J. Ballet, I. Sermet-Gaudelus, 2016, Journal of Clinical Microbiology.

A large subset of neutrophils expressing membrane proteinase 3 is a risk factor for vasculitis and rheumatoid arthritis.

B. Prum, J. Timsit, J. Grünfeld, 1999, Journal of the American Society of Nephrology : JASN.

Diagnosis of Cystic Fibrosis: Consensus Guidelines from the Cystic Fibrosis Foundation

Margaret Rosenfeld, Frank Accurso, Philip M. Farrell, 2017, The Journal of pediatrics.

Achromobacter xylosoxidans airway infection is associated with lung disease severity in children with cystic fibrosis

I. Sermet-Gaudelus, G. Thouvenin, H. Corvol, 2021, ERJ Open Research.

Basic protocol for transepithelial nasal potential difference measurements.

Michael R Knowles, Jean Lebacq, Patrick Lebecque, 2004, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Measurement of immunoglobulin G against Mycobacterial antigen A60 in patients with cystic fibrosis and lung infection due to Mycobacterium abscessus.

P. Berche, I. Sermet-Gaudelus, J. Gaillard, 2005, Clinical infectious diseases : an official publication of the Infectious Diseases Society of America.

Bothnian Palmoplantar Keratoderma: Further Delineation of the Associated Phenotype

S. Hadj-Rabia, J. Steffann, I. Sermet-Gaudelus, 2022, Genes.

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

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

Mutations in GAS8, a Gene Encoding a Nexin‐Dynein Regulatory Complex Subunit, Cause Primary Ciliary Dyskinesia with Axonemal Disorganization

S. Amselem, A. Clément, I. Sermet-Gaudelus, 2016, Human mutation.

European Cystic Fibrosis Society Standards of Care: Best Practice guidelines.

Sarah Jane Schwarzenberg, S. Bell, F. Ratjen, 2014, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Down-regulation of the Anti-inflammatory Protein Annexin A1 in Cystic Fibrosis Knock-out Mice and Patients*S

F. Halgand, M. Perretti, I. Sermet-Gaudelus, 2005, Molecular & Cellular Proteomics.

Proteomic profiling of sweat in patients with cystic fibrosis provides new insights into epidermal homoeostasis

I. Sermet-Gaudelus, T. Nguyen-Khoa, Vincent Jung, 2022, Skin health and disease.

Characterization of SLC26A9 in Patients with CF‐Like Lung Disease

Annick Thomas, N. Farman, S. Amselem, 2013, 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.

Therapeutic pipeline for individuals with cystic fibrosis with mutations nonresponsive to current cystic fibrosis transmembrane conductance regulator modulators

I. Sermet-Gaudelus, I. Fajac, 2021, Current opinion in pulmonary medicine.

CFTR: New insights into structure and function and implications for modulation by small molecules.

J. Hunt, I. Sermet-Gaudelus, I. Callebaut, 2019, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Correction to: In vitro prediction of stop-codon suppression by intravenous gentamicin in patients with cystic fibrosis: a pilot study

J. Rousset, L. Bidou, I. Sermet-Gaudelus, 2018, BMC Medicine.

New Pharmacological Approaches to Treat Patients with Cystic Fibrosis with Nonsense Mutations.

O. Namy, I. Sermet-Gaudelus, 2016, American journal of respiratory and critical care medicine.

Guidelines on the early management of infants diagnosed with cystic fibrosis following newborn screening.

I. Sermet-Gaudelus, K. Southern, S. Mayell, 2010, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

β‐adrenergic sweat test in children with inconclusive cystic fibrosis diagnosis: Do we need new reference ranges?

H. Zar, I. Sermet-Gaudelus, T. Gonska, 2022, Pediatric pulmonology.

Plasma lipidomics reveals potential prognostic signatures within a cohort of cystic fibrosis patients

Daniele Piomelli, Giuseppe Astarita, D. Piomelli, 2011, Journal of Lipid Research.

Cystic Fibrosis Diagnosis in Newborns, Children, and Adults

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

European best practice guidelines for cystic fibrosis neonatal screening.

O. Sommerburg, M. Macek, C. Férec, 2009, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Pharmacokinetic and Pharmacodynamic Optimization of Antibiotic Therapy in Cystic Fibrosis Patients: Current Evidences, Gaps in Knowledge and Future Directions

I. Sermet-Gaudelus, V. Jullien, M. Launay, 2021, Clinical Pharmacokinetics.

Simple pediatric nutritional risk score to identify children at risk of malnutrition.

C. Ricour, I. Sermet-Gaudelus, V. Colomb, 2000, The American journal of clinical nutrition.

Multifocal fixed drug eruption to ceftazidime in a child with cystic fibrosis

I. Sermet-Gaudelus, N. Bellon, J. de Blic, 2018, Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology.

rAAV-CFTRΔR Rescues the Cystic Fibrosis Phenotype in Human Intestinal Organoids and Cystic Fibrosis Mice.

V. Baekelandt, M. Ferrante, A. Ramalho, 2016, American journal of respiratory and critical care medicine.

Multi-physiopathological consequences of the c.1392G>T CFTR mutation revealed by clinical and cellular investigations.

A. Kitzis, I. Sermet-Gaudelus, E. Bieth, 2014, Biochemistry and cell biology = Biochimie et biologie cellulaire.

Genotypic and Phenotypic Variation in Pseudomonas aeruginosa Reveals Signatures of Secondary Infection and Mutator Activity in Certain Cystic Fibrosis Patients with Chronic Lung Infections

Scott R. Miller, F. Taddei, I. Sermet-Gaudelus, 2011, Infection and Immunity.

Lung resection in cystic fibrosis: A survival analysis

P. Camargos, Y. Révillon, I. Sermet-Gaudelus, 2008, Pediatric pulmonology.

Exon identity influences splicing induced by exonic variants and in silico prediction efficacy.

I. Sermet-Gaudelus, E. Girodon, J. Varilh, 2020, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Priming of blood neutrophils in children with cystic fibrosis: correlation between functional and phenotypic expression of opsonin receptors before and after platelet-activating factor priming.

R. Allen, I. Sermet-Gaudelus, B. Descamps-Latscha, 1999, The Journal of infectious diseases.

[Body temperature measurement in daily practice].

I. Sermet-Gaudelus, G. Lenoir, I. Chadelat, 2005, Archives de pediatrie : organe officiel de la Societe francaise de pediatrie.

Overexpression of RANKL in osteoblasts: a possible mechanism of susceptibility to bone disease in cystic fibrosis

I. Sermet-Gaudelus, C. Guillaume, S. Gangloff, 2016, The Journal of pathology.

Genetic deletion of keratin 8 corrects the altered bone formation and osteopenia in a mouse model of cystic fibrosis.

K. Zatloukal, E. Laplantine, V. Geoffroy, 2016, Human molecular genetics.

Cystic fibrosis bone disease: is the CFTR corrector C18 an option for therapy?

L. Touqui, F. Le Pimpec Barthès, I. Sermet-Gaudelus, 2014, European Respiratory Journal.

European cystic fibrosis bone mineralisation guidelines.

J. Compston, I. Sermet-Gaudelus, C. Haworth, 2011, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

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.

Prevention of osteoporosis in cystic fibrosis.

I. Sermet-Gaudelus, F. Chédevergne, 2019, Current opinion in pulmonary medicine.

Lessons from a French collaborative case–control study in cystic fibrosis patients during the 2009 A/H1N1 influenza pandemy

P. Boelle, I. Durieu, N. Stremler, 2015, BMC Infectious Diseases.

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.

Continuous versus Intermittent Infusions of Ceftazidime for Treating Exacerbation of Cystic Fibrosis

C. Cracowski, B. Wallaert, I. Sermet-Gaudelus, 2009, Antimicrobial Agents and Chemotherapy.

Persistent Bordetella bronchiseptica infection in a child with cystic fibrosis: Relationship to bacterial phenotype.

I. Sermet-Gaudelus, A. Ferroni, N. Guiso, 2015, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Long-term safety and efficacy of ivacaftor in patients with cystic fibrosis who have the Gly551Asp-CFTR mutation: a phase 3, open-label extension study (PERSIST).

Ying Jiang, Barry Plant, Matthias Griese, 2014, The Lancet. Respiratory medicine.

December 2002 Fellowships Grants and Awards

I. Sermet-Gaudelus, B. Descamps-Latscha, G. Lenoir, 2002, Environmental Health Perspectives.

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.

Insights Into Patient Variability During Ivacaftor-Lumacaftor Therapy in Cystic Fibrosis

T. Velkov, M. Griese, I. Sermet-Gaudelus, 2021, Frontiers in Pharmacology.

Pseudomonas aeruginosa LasB protease impairs innate immunity in mice and humans by targeting a lung epithelial cystic fibrosis transmembrane regulator–IL-6–antimicrobial–repair pathway

A. Cazes, J. Sallenave, I. Sermet-Gaudelus, 2017, Thorax.

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.

The French compassionate programme of elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis with advanced lung disease and no F508del CFTR variant

P. Burgel, I. Durieu, I. Sermet-Gaudelus, 2023, European Respiratory Journal.

Lumacaftor-ivacaftor effects on cystic fibrosis-related liver involvement in adolescents with homozygous F508 del-CFTR.

I. Sermet-Gaudelus, E. Schneider-Futschik, Jérémy Dana, 2021, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Acute tubulointerstitial nephritis with or without uveitis: a novel form of post-acute COVID-19 syndrome in children

J. Casanova, J. Bacchetta, B. Ranchin, 2023, medRxiv.

Normal Function of the Cystic Fibrosis Conductance Regulator Protein Can Be Associated with Homozygous ΔF508 Mutation

J. Bernaudin, I. Sermet-Gaudelus, J. Bresson, 2002, Pediatric Research.

Elexacaftor/Tezacaftor/Ivacaftor Disrupts Respiratory Tract Development in a Murine Fetal Lung Explant Model

C. Delacourt, Nicolas Houde, I. Sermet-Gaudelus, 2022, American journal of respiratory cell and molecular biology.

US Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus recommendations for the management of non-tuberculous mycobacteria in individuals with cystic fibrosis

R. Gibson, L. Saiman, P. Corris, 2015, Thorax.

Effect of ivacaftor treatment in patients with cystic fibrosis and the G551D-CFTR mutation: patient-reported outcomes in the STRIVE randomized, controlled trial

M. Bayliss, A. Quittner, I. Sermet-Gaudelus, 2015, Health and Quality of Life Outcomes.

Airway Surface Liquid pH Regulation in Airway Epithelium Current Understandings and Gaps in Knowledge

I. Sermet-Gaudelus, G. Planelles, E. Dreano, 2021, International journal of molecular sciences.

Impact of COVID-19 on people with cystic fibrosis

P. Burgel, I. Sermet-Gaudelus, K. Southern, 2020, The Lancet Respiratory Medicine.

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.

Prior infection by seasonal coronaviruses, as assessed by serology, does not prevent SARS-CoV-2 infection and disease in children, France, April to June 2020

D. Kariyawasam, C. Huon, I. Sermet-Gaudelus, 2021, Euro surveillance : bulletin Europeen sur les maladies transmissibles = European communicable disease bulletin.

Chronic Staphylococcus aureus lung infection correlates with proteogenomic and metabolic adaptations leading to an increased intracellular persistence

A. Charbit, I. Guerrera, X. Nassif, 2018, bioRxiv.

Pain is an underestimated symptom in cystic fibrosis

I. Sermet-Gaudelus, M. Kirszenbaum, A. Masson, 2017, Current opinion in pulmonary medicine.

Clinical phenotype and genotype of children with borderline sweat test and abnormal nasal epithelial chloride transport.

Milan Macek, Gérard Lenoir, J. Sarles, 2010, American journal of respiratory and critical care medicine.

Bacterial contamination in the environment of hospitalised children with cystic fibrosis.

P. Berche, I. Sermet-Gaudelus, G. Lenoir, 2008, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Involvement of CFTR in the pathogenesis of pulmonary arterial hypertension

M. Humbert, C. Péchoux, P. Burgel, 2021, European Respiratory Journal.

Comparative proteomics of respiratory exosomes in cystic fibrosis, primary ciliary dyskinesia and asthma.

I. Guerrera, I. Sermet-Gaudelus, A. Schmitt, 2018, Journal of proteomics.

Arterial abnormalities identified in kidneys transplanted into children during the COVID-19 pandemic

N. Boddaert, N. Garcelon, S. Sarnacki, 2020, American Journal of Transplantation.

Infections caused by Mycobacterium abscessus: epidemiology, diagnostic tools and treatment

I. Sermet-Gaudelus, N. Veziris, E. Cambau, 2016, Expert review of anti-infective therapy.

Representation and quantification Of Module Activity from omics data with rROMA

L. Calzone, L. Martignetti, A. Zinovyev, 2022, bioRxiv.

Inflammation and CFTR: might neutrophils be the key in cystic fibrosis?

I. Sermet-Gaudelus, B. Descamps-Latscha, G. Lenoir, 1999, Mediators of inflammation.

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.

Characterization of Nasal Potential Difference in cftr Knockout and F508del-CFTR Mice

I. Sermet-Gaudelus, A. Edelman, L. Debarbieux, 2013, PloS one.

Disruption of cytokeratin-8 interaction with F508del-CFTR corrects its functional defect.

G. Lukács, I. Guerrera, I. Sermet-Gaudelus, 2012, Human molecular genetics.

Unsolved severe chronic rhinosinusitis elucidated by extensive CFTR genotyping

S. Simon, I. Sermet-Gaudelus, I. Callebaut, 2019, Clinical case reports.

25 Unsolved severe chronic rhinosinusitis elucidated by extensive CFTR genotyping

S. Simon, I. Sermet-Gaudelus, I. Callebaut, 2017 .

Changes of CFTR functional measurements and clinical improvements in cystic fibrosis patients with non p.Gly551Asp gating mutations treated with ivacaftor.

I. Sermet-Gaudelus, M. Wilschanski, T. Nguyen-Khoa, 2017, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Effect of mutator P. aeruginosa on antibiotic resistance acquisition and respiratory function in cystic fibrosis

F. Taddei, P. Berche, I. Sermet-Gaudelus, 2009, Pediatric pulmonology.

Severe COVID‐19 evolving towards organizing pneumonia in a pediatric lung transplant recipient

P. Frange, I. Sermet-Gaudelus, D. Drummond, 2021, Pediatric pulmonology.

Identification of non fermenting Gram negative bacilli isolated in cystic fibrosis by Matrix assisted laser desorption ionization time-of-flight mass spectrometry 1 2 3 4 5 6 7 8 9 10 11 12 13

P. Berche, X. Nassif, I. Sermet-Gaudelus, 2008 .

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

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

Cystic fibrosis transmembrane conductance regulator channel dysfunction in non-cystic fibrosis bronchiectasis.

P. Burgel, I. Sermet-Gaudelus, I. Fajac, 2010, American journal of respiratory and critical care medicine.

Lung-adapted Staphylococcus aureus isolates with dysfunctional agr system trigger a proinflammatory response.

A. Charbit, X. Nassif, I. Sermet-Gaudelus, 2022, The Journal of infectious diseases.

Standardized Measurement of Nasal Membrane Transepithelial Potential Difference (NPD).

I. Sermet-Gaudelus, S. Rowe, P. Melotti, 2018, Journal of visualized experiments : JoVE.

A Novel Lipidomic Strategy Reveals Plasma Phospholipid Signatures Associated with Respiratory Disease Severity in Cystic Fibrosis Patients

J. Jais, D. Piomelli, G. Astarita, 2009, PloS one.

P265 Real-life initiation of lumacaftor/ivacaftor in adolescents and adults homozygous for the F508del CFTR mutation: a French nationwide study

P. Reix, P. Burgel, I. Durieu, 2019, Journal of Cystic Fibrosis.

Factors associated with humoral immune response to pandemic A/H1N1(v) 2009 influenza vaccine in cystic fibrosis.

P. Böelle, P. Boelle, I. Sermet-Gaudelus, 2014, Vaccine.

Genomic analysis of Staphylococcus aureus sequential isolates from lungs of patients with cystic fibrosis.

A. Charbit, I. Sermet-Gaudelus, A. Ferroni, 2023, Microbes and infection.

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.

Omalizumab treatment for allergic bronchopulmonary aspergillosis in young patients with cystic fibrosis.

A. Clément, I. Sermet-Gaudelus, J. Derelle, 2017, Respiratory medicine.

Rapid Improvement of Intracranial Tuberculomas After Addition of Ofloxacin to First-Line Antituberculosis Treatment

I. Sermet-Gaudelus, J. Gaillard, G. Lenoir, 1999, European Journal of Clinical Microbiology and Infectious Diseases.

Suppressing ‘nonsense’ in cystic fibrosis

I. Sermet-Gaudelus, D. Sheppard, A. Hinzpeter, 2019, Journal of Physiology.

Persistent Staphylococcus aureus infections in children with chronic lung disease: a multi-omics analysis of bacterial adaptation to define novel therapeutic approaches

A. Charbit, I. Guerrera, X. Nassif, 2018 .

Antisense oligonucleotide eluforsen improves CFTR function in F508del cystic fibrosis

J. Clancy, J. Bolognese, J. Nick, 2018, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Hereditary Hypophosphatemic Rickets with Hypercalciuria: Report of a New Kindred

I. Sermet-Gaudelus, G. Lenoir, M. Déchaux, 2001, Nephron.

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

O. Namy, L. Bidou, I. Sermet-Gaudelus, 2023, bioRxiv.

[Novel therapies for cystic fibrosis in 2009].

I. Sermet-Gaudelus, G. Lenoir, A. Edelman, 2009, Archives de pediatrie : organe officiel de la Societe francaise de pediatrie.

Vaccine coverage in CF children: A French multicenter study.

I. Sermet-Gaudelus, O. Launay, H. Corvol, 2015, Journal of Cystic Fibrosis.

US Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus recommendations for the management of non-tuberculous mycobacteria in individuals with cystic fibrosis: executive summary

R. Gibson, L. Saiman, P. Corris, 2015, Thorax.

Continuous versus Intermittent Infusions of Ceftazidime for Treating Exacerbation of Cystic Fibrosis

C. Cracowski, B. Wallaert, I. Sermet-Gaudelus, 2009 .

Penetrance is a critical parameter for assessing the disease liability of CFTR variants.

C. Férec, I. Sermet-Gaudelus, M. Audrézet, 2020, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Journal of Cystic Fibrosis

A. Kraneveld, C. Castellani, I. Sermet-Gaudelus, 2022 .

Representation and quantification Of Module Activity from omics data with rROMA

L. Martignetti, A. Zinovyev, I. Sermet-Gaudelus, 2023, bioRxiv.

Moving the Dial on Airway Inflammation in Response to Trikafta in Adolescents with Cystic Fibrosis

P. Reix, E. Kerem, I. Sermet-Gaudelus, 2023, American journal of respiratory and critical care medicine.

[Focus on cystic fibrosis].

I. Sermet-Gaudelus, Marlène Clairicia Pierre-Joseph, A. Lustre, 2020, Revue de l'infirmiere.

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.

US Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus recommendations for the management of non-tuberculous mycobacteria in individuals with cystic fibrosis

L. Saiman, I. Sermet-Gaudelus, J. Herrmann, 2015, Thorax.

The hsa-miR-125a/hsa-let-7e/hsa-miR-99b cluster is potentially implicated in Cystic Fibrosis pathogenesis.

L. Mazzolini, I. Sermet-Gaudelus, T. Bienvenu, 2015, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Title : 2 Persistent Staphylococcus aureus infections in children with 3 chronic lung disease : a multi-omics analysis of bacterial adaptation 4 to define novel therapeutic approaches

A. Charbit, I. Guerrera, X. Nassif, 2022 .

ECFS best practice guidelines: the 2018 revision.

C. Castellani, P. Dřevínek, S. Bell, 2018, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Factors associated with clinical progression to severe COVID-19 in people with cystic fibrosis: A global observational study

C. Goss, H. Rodgers, P. Burgel, 2022, Journal of Cystic Fibrosis.

Pain in children and adults with cystic fibrosis: a comparative study.

I. Sermet-Gaudelus, G. Lenoir, D. Dusser, 2009, Journal of pain and symptom management.

December 2002 Fellowships Grants and Awards

I. Sermet-Gaudelus, B. Descamps-Latscha, G. Lenoir, 2002, Environmental Health Perspectives.

Clinical changes of patients with cystic fibrosis during transition from pediatric to adult care.

I. Sermet-Gaudelus, M. Le Bourgeois, N. Desmazes-Dufeu, 2008, The Journal of adolescent health : official publication of the Society for Adolescent Medicine.