A. Hinzpeter

发表

The Autophagy Inhibitor Spautin-1 Antagonizes Rescue of Mutant CFTR Through an Autophagy-Independent and USP13-Mediated Mechanism

D. di Bernardo, N. Pedemonte, L. Galietta, 2018, Front. Pharmacol..

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

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

Analysis of nasal potential in murine cystic fibrosis models.

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

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

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

Blue Native/SDS-PAGE Analysis Reveals Reduced Expression of the mClCA3 Protein in Cystic Fibrosis Knock-out Mice*

A. Gruber, A. Edelman, M. Ollero, 2005, Molecular & Cellular Proteomics.

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.

Factors influencing readthrough therapy for frequent cystic fibrosis premature termination codons

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

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.

COMMD1 modulates noxious inflammation in cystic fibrosis.

S. Moutéreau, A. Hinzpeter, S. le Gouvello, 2013, The international journal of biochemistry & cell biology.

Alternative Splicing of In‐Frame Exon Associated with Premature Termination Codons: Implications for Readthrough Therapies

L. Galietta, E. Girodon, E. Bieth, 2013, Human mutation.

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.

Targeting different binding sites in the CFTR structures allows to synergistically potentiate channel activity.

J. Mornon, I. Callebaut, P. Lehn, 2020, European journal of medicinal chemistry.

Effect of ouabain on CFTR gene expression in human Calu-3 cells.

A. Edelman, A. Hinzpeter, F. Brouillard, 2003, American journal of physiology. Cell physiology.

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.

Evidence for direct CFTR inhibition by CFTRinh-172 based on arginine 347 mutagenesis

R. Ravazzolo, A. Verkman, N. Sonawane, 2020 .

Evidence for direct CFTR inhibition by CFTR(inh)-172 based on Arg347 mutagenesis.

R. Ravazzolo, A. Verkman, N. Sonawane, 2008, The Biochemical journal.

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.

Genetics of cystic fibrosis: CFTR mutation classifications toward genotype-based CF therapies.

A. Hinzpeter, Alexandre Hinzpeter, Pascale Fanen, 2014, The international journal of biochemistry & cell biology.

CSN5 binds to misfolded CFTR and promotes its degradation.

M. Goossens, N. Arous, A. Hinzpeter, 2008, Biochimica et biophysica acta.

Design of crotoxin-based peptides with potentiator activity targeting the ΔF508NBD1 Cystic Fibrosis Transmembrane Conductance Regulator.

P. Corringer, G. Lukács, P. Zielenkiewicz, 2022, Journal of molecular biology.

Combined Computational–Experimental Analyses of CFTR Exon Strength Uncover Predictability of Exon‐Skipping Level

E. Girodon, C. Vasseur, M. Goossens, 2013, Human mutation.

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.

A recurrent deep-intronic splicing CF mutation emphasizes the importance of mRNA studies in clinical practice.

M. Claustres, R. Epaud, E. Girodon, 2011, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

COMMD1-Mediated Ubiquitination Regulates CFTR Trafficking

M. Goossens, N. Arous, A. Hinzpeter, 2011, PloS one.

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.

part of transcripts UTR ' expression via influence of MAPK cascades on 3 Cell-specific posttranscriptional regulation of CFTR gene

A. Edelman, A. Hinzpeter, B. Costes, 2016 .

Cell-specific posttranscriptional regulation of CFTR gene expression via influence of MAPK cascades on 3'UTR part of transcripts.

A. Edelman, A. Hinzpeter, F. Brouillard, 2005, American journal of physiology. Cell physiology.

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.

Alternative Splicing at a NAGNAG Acceptor Site as a Novel Phenotype Modifier

Bruno Costes, E. Girodon, M. Goossens, 2010, PLoS genetics.

Mutation-Specific Potency and Efficacy of Cystic Fibrosis Transmembrane Conductance Regulator Chloride Channel Potentiators

N. Pedemonte, A. Caputo, N. Arous, 2009, Journal of Pharmacology and Experimental Therapeutics.

Biochemical methods to assess CFTR expression and membrane localization.

G. Lukács, M. Amaral, C. Farinha, 2004, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

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.

The influence of CFTR complex alleles on precision therapy of cystic fibrosis.

A. Hinzpeter, B. Chevalier, 2019, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

CFTR corrector efficacy is associated with occupancy of distinct binding sites

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

The functioning of mammalian ClC-2 chloride channel in Saccharomyces cerevisiae cells requires an increased level of Kha1p.

A. Edelman, A. Hinzpeter, A. Kurlandzka, 2005, The Biochemical journal.

Membrane Cholesterol Content Modulates ClC-2 Gating and Sensitivity to Oxidative Stress*

A. Edelman, M. Ollero, J. Clain, 2007, Journal of Biological Chemistry.

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.

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 .

The importance of functional tests to assess the effect of a new CFTR variant when genotype–phenotype correlation is not possible

P. Fergelot, D. Lacombe, I. Callebaut, 2017, Clinical case reports.

8 The importance of functional tests to assess the effect of a new CFTR variant when genotype-phenotype correlation is not possible

P. Fergelot, D. Lacombe, E. Girodon, 2016 .

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

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

Immunohistochemistry of CFTR in native tissues and primary epithelial cell cultures.

C. Férec, M. Amaral, D. Penque, 2004, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

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.

Suppressing ‘nonsense’ in cystic fibrosis

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

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

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

Genetics of cystic fibrosis: CFTR mutation classifications toward genotype-based CF therapies.

A. Hinzpeter, P. Fanen, Adeline Wohlhuter-Haddad, 2014, The international journal of biochemistry & cell biology.

Association between Hsp90 and the ClC-2 chloride channel upregulates channel function.

M. Dadlez, A. Edelman, J. Lipecka, 2006, American journal of physiology. Cell physiology.

Genetics of cystic fibrosis: CFTR mutation classifications toward genotype-based CF therapies.

A. Hinzpeter, P. Fanen, Adeline Wohlhuter-Haddad, 2014, The international journal of biochemistry & cell biology.

[Therapeutics in cystic fibrosis: Clinical revolution and new challenges].

A. Hinzpeter, I. Sermet-Gaudelus, Emmanuelle Bardin, 2024, Medecine sciences : M/S.