25 Unsolved severe chronic rhinosinusitis elucidated by extensive CFTR genotyping von Degrugillier, F, Simon, S, Remus, N, Mekki, C, Bassinet, L, Decrouy, X, Sermet-Gaudelus, I, Hatton, A, Hoffmann, B, Hinzpeter, A, Callebaut, I, Fanen, P, Prulière-Escabasse, V

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Pharmacological chaperones improve intra-domain stability and inter-domain assembly via distinct binding sites to rescue misfolded CFTR von Baatallah, Nesrine, Elbahnsi, Ahmad, Mornon, Jean-Paul, Chevalier, Benoit, Pranke, Iwona, Servel, Nathalie, Zelli, Renaud, Décout, Jean-Luc, Edelman, Aleksander, Sermet-Gaudelus, Isabelle, Callebaut, Isabelle, Hinzpeter, Alexandre

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8 The importance of functional tests to assess the effect of a new CFTR variant when genotype-phenotype correlation is not possible von Hinzpeter, A, Reboul, M.-P, Zordan, C, Costes, B, Guichoux, J, Iron, A, Lacombe, D, Martin, N, Arveiler, B, Fanen, P, Fergelot, P, Girodon, E

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Memantine-associated Reversal of Clozapine-induced Weight Gain von Schaefer, M., Leopold, K., Hinzpeter, A., Heinz, A., Krebs, M.

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WS21.6 A novel 5′ alternative CFTR mRNA isoform may be a cause of CFTR dysfunction in a patient with nasal polyposis von Hinzpeter, A, de Becdelièvre, A, Bieth, E, Gameiro, C, Brémont, F, Martin, N, Costes, B, Costa, C, Aissat, A, Goossens, M, Fanen, P, Girodon, E

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The expanded French compassionate programme for elexacaftor–tezacaftor–ivacaftor use in people with cystic fibrosis without a F508del CFTR variant: a real-world study von Burgel, Pierre-Régis, Sermet-Gaudelus, Isabelle, Girodon, Emmanuelle, Durieu, Isabelle, Houdouin, Véronique, Audousset, Camille, Macey, Julie, Grenet, Dominique, Porzio, Michele, Murris-Espin, Marlène, Reix, Philippe, Baravalle, Mélisande, Belleguic, Chantal, Mely, Laurent, Verhille, Juliette, Weiss, Laurence, Reynaud-Gaubert, Martine, Mittaine, Marie, Hamidfar, Rebecca, Ramel, Sophie, Cosson, Laure, Danner-Boucher, Isabelle, Foucaud, Pierre, Roy, Charlotte, Burnet, Espérie, Raynal, Caroline, Audrezet, Marie-Pierre, Da Silva, Jennifer, Martin, Clémence, Kanaan, Reem, Carlier, Nicolas, Honoré, Isabelle, Chedevergne, Frédérique, Dreano, Elise, Hatton, Aurélie, Hinzpeter, Alexandre, Pranke, Iwona, Le Clainche-Viala, Laurence, Mayer, Sophie, Corvol, Harriet, Thouvenin, Guillaume, de Miranda, Sandra, Remus, Natascha, Duthoit, Louise, Perez, Thierry, Wizla, Nathalie, Bon, Claire, Bui, Stéphanie, Poey, Nora, Stremler, Nathalie, Coltey, Bérengère, Dufeu, Nadine, Lebihan, Jean, Gabsi, Asma, Pouradier, Delphine, Andrejak, Claire, Rames, Cinthia, Dupuy-Grasset, Magali, Languepin, Jeanne, Marguet, Christophe, Pramil, Stéphanie, Arnouat, Baptiste, Fanton, Annlyse, Abely, Michel, Ravoninjatovo, Bruno, Blondé, Aurore, Guillaumot, Anne, Kieffer, Sebastien, Tatopoulos, Aurélie, Nove-Josserand, Raphaële, Ohlmann, Camille, Perrin, Thomas, Reynaud, Quitterie, Llerena, Catherine, Quétant, Sébastien, Valois, Sophie, Dalphin, Marie-Laure, Richaud-Thiriez, Bénédicte, Deneuville, Eric, Chiron, Raphael, Socchi, Floriane, Bihouée, Tiphaine, Mankikian, Julie, Flament, Thomas, Coolen-Allou, Nathalie, Gachelin, Elsa, Périsson, Caroline, Vuillard, Constance, Alkoussa, Wael, Marchal, Sarah, Leroy, Sylvie, Campbell, Karine, Laurans, Muriel, Labbé, Guillaume, Montcouquiol, Sylvie, Priou, Pascaline, de Carli, Paola, Lemonnier, Lydie, Dehillotte, Clémence, Nouvel, Thierry

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351 Beyond elexacaftor/tezacaftor/ivacaftor: mechanistic insights to maximize N1303K-CFTR rescue von Pranke, I., Capurro, V., Chevalier, B., Pesce, E., Tomati, V., Pastorino, C., Hatton, A., Dreano, E., Kelly-Aubert, M., Guerrera, C., Galietta, L., Girodon, E., Burgel, P., Sermet-Gaudelus, I., Hinzpeter, A., Pedemonte, N.

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689 Pharmacological chaperones improve intradomain stability and interdomain assembly via distinct binding sites to rescue misfolded cystic fibrosis transmembrane conductance regul... von Baatallah, N., Elbahnsi, A., Mornon, J., Chevalier, B., Pranke, I., Servel, N., Zelli, R., Décout, J., Edelman, A., Sermet, I., Callebaut, I., Hinzpeter, A.

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673 Elexacaftor/tezacaftor/ivacaftor corrects function of H1085R-, N1303K-, and R334W-cystic fibrosis transmembrane conductance regulator and improves clinical status of patients von Pranke, I., Dreano, E., Hatton, A., Lepissier, A., Hinzpeter, A., Martin, C., Le Bihan, J., Burgel, P., Durieu, I., Kanaan, R., de Carli, P., Sermet, I.

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In vivo and in vitro effect of E831X mutation von Hinzpeter, A, Costa, C, Alembik, Y, Weiss, L, Goossens, M, Girodon, E, Fanen, P

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Role of COMMD1 and CSN5 in transcriptional regulation of the CFTR gene von de Becdelièvre, A, Aissat, A, Drévillon, L, Hinzpeter, A, Arous, N, Fanen, P

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COMMD1 promotes CFTR trafficking through inhibition of ubiquitination von Drévillon, L, Tanguy, G, Saubamea, B, de Becdelièvre, A, Hinzpeter, A, Arous, N, Fanen, P

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Exonic sequence variations affecting splicing within exons 3, 4 and 5 von Hinzpeter, A, Aissat, A, Golmard, L, de Becdelièvre, A, Costes, B, Goossens, M, Girodon, E, Costa, C, Fanen, P

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WS05.1 AVX-770 binding site within CFTR membrane spanning domain 2 enables ATP-independent channel activation von Baatallah, N., Elbahnsi, A., Mornon, J.-P., Chevalier, B., Pranke, I., Servel, N., Edelman, A., Sermet-Gaudelus, I., Callebaut, I., Hinzpeter, A.

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Prevention of interferon-alpha associated depression in psychiatric risk patients with chronic hepatitis C von Schaefer, Martin, Schwaiger, Markus, Garkisch, Andrea S., Pich, Maurice, Hinzpeter, Axel, Uebelhack, Ralf, Heinz, Andreas, van Boemmel, Florian, Berg, Thomas

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Combined Computational-Experimental Analyses of CFTR Exon Strength Uncover Predictability of Exon-Skipping Level von Aissat, Abdel, de Becdelièvre, Alix, Golmard, Lisa, Vasseur, Christian, Costa, Catherine, Chaoui, Asma, Martin, Natacha, Costes, Bruno, Goossens, Michel, Girodon, Emmanuelle, Fanen, Pascale, Hinzpeter, Alexandre

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The functioning of mammalian CIC-2 chloride channel in Saccharomyces cerevisiae cells requires an increased level of Kha1p von Flis, K, Hinzpeter, A, Edelman, A, Kurlandzka, A

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Blue Native/SDS-PAGE Analysis Reveals Reduced Expression of the mClCA3 Protein in Cystic Fibrosis Knock-out Mice von Brouillard, Franck, Bensalem, Noura, Hinzpeter, Alexandre, Tondelier, Danielle, Trudel, Stéphanie, Gruber, Achim D, Ollero, Mario, Edelman, Aleksander

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Association between Hsp90 and the ClC-2 chloride channel upregulates channel function von Hinzpeter, Alexandre, Lipecka, Joanna, Brouillard, Franck, Baudoin-Legros, Maryvonne, Dadlez, Michal, Edelman, Aleksander, Fritsch, Janine

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Cell-specific posttranscriptional regulation of CFTR gene expression via influence of MAPK cascades on 3′UTR part of transcripts von Baudouin-Legros, Maryvonne, Hinzpeter, Alexandre, Jaulmes, Amandine, Brouillard, Franck, Costes, Bruno, Fanen, Pascale, Edelman, Aleksander

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