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I

Iwona Pranke

Institut Necker Enfants Malades
H指数:18
被引数:1,330

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Beyond Trikafta: new models to assess tissue dependent rescue of N1303K-CFTR

超越Trikafta：评估组织依赖性N1303K-CFTR挽救的新模型

Frontiers in Pharmacology
IF4.8
2025-10-290
OAAI
Iwona Pranke†; Valeria Capurro†; Benoit Chevalier†; Emanuela Pesce†; Valeria Tomati; Cristina Pastorino; Mairead Kelly-Aubert; Aurelie Hatton; Elise Dreano; Mariateresa Lena; Renata Bocciardi; Federico Zara; Stefano Pantano; Vito Terlizzi; Cristina Lucanto; Stefano Costa; Laura Claut; Valeria Daccò; Piercarlo Poli; Massimo Maschio; Benedetta Fabrizzi; Nicole Caporelli; Marco Cipolli; Sonia Volpi; Frederique Chedevergne; Laure Cosson; Julie Macey; Sophie Ramel; Laurence Weiss; Dominique Grenet; Laurence Le Clainche-Viala; Benoit Douvry; Bruno Ravoninjatovo; Camille Audousset; Aurélie Tatopoulos; Bénédicte Richaud-Thiriez; Melissa Baravalle; Guillaume Thouvenin; Guillaume Labbé; Marie Mittaine; Philippe Reix; Isabelle Durieu; Julie Mankikian; Stéphanie Bui; Thao Nguyen-Khoa; Karim Khoukh; Clémence Martin; Jennifer Da Silva; Paola De Carli; Carlo Castellani; Federico Cresta; Luis Galietta; Anne Guillemaut; Emmanuelle Girodon; Natacha Remus; Mathis Bulcaen; Marjolein Ensinck; Miroslaw Zajac; Marianne Carlon; Jean LeBihan; Pierre-Régis Burgel; Isabelle Sermet-Gaudelus‡; Alexandre Hinzpeter‡; Nicoletta Pedemonte‡
Adenine base editing with engineered virus-like particles rescues the CFTR mutation G542X in patient-derived intestinal organoids

使用工程化类病毒颗粒进行的腺嘌呤碱基编辑在患者来源的肠类器官中修复了CFTR基因的G542X突变

ISCIENCE
IF4.1
2025-02-010
OAAI
Nicosia, Lucia; Pranke, Iwona; Latorre, Roberta V.; Murray, Joss B.; Lonetti, Lisa; Cavusoglu-Doran, Kader; Dreano, Elise; Costello, James P.; Carroll, Michael; Melotti, Paola; Sorio, Claudio; Sermet-Gaudelus, Isabelle; Scallan, Martina F.; Harrison, Patrick T.
A small molecule chaperone rescues keratin-8 mediated trafficking of misfolded podocin to correct genetic Nephrotic Syndrome

小分子伴侣可挽救角蛋白8介导的错误折叠的podocin运输，以纠正遗传性肾病综合征

KIDNEY INTERNATIONAL
IF12.6
2024-04-012
OA
Kuzmuk, Valeryia; Pranke, Iwona; Rollason, Ruth; Butler, Matthew; Ding, Wen Y.; Beesley, Matthew; Waters, Aoife M.; Coward, Richard J.; Sessions, Richard; Tuffin, Jack; Foster, Rebecca R.; Mollet, Geraldine; Antignac, Corinne; Edelman, Aleksander; Welsh, Gavin I.; Saleem, Moin A.
Nonsense mutations accelerate lung disease and decrease survival of cystic fibrosis children
JOURNAL OF CYSTIC FIBROSIS
IF6
2023-11-011
PRE
Orenti, Annalisa; Pranke, Iwona; Faucon, Caroline; Varilh, Jessica; Hatton, Aurelie; Golec, Anita; Dehillotte, Clemence; Durieu, Isabelle; Reix, Philippe; Burgel, Pierre-Regis; Grenet, Dominique; Tasset, Celine; Gachelin, Elsa; Perisson, Caroline; Lepissier, Agathe; Dreano, Elise; Tondelier, Danielle; Chevalier, Benoit; Weiss, Laurence; Kiefer, Sebastien; Laurans, Muriel; Chiron, Raphael; Lemonnier, Lydie; Marguet, Christophe; Jung, Andreas; Edelman, Aleksander; Kerem, Bat-Sheva; Girodon, Emmanuelle; Taulan-Cadars, Magali; Hinzpeter, Alexandre; Kerem, Eitan; Naehrlich, Lutz; Sermet-Gaudelus, Isabelle
Theratyping cystic fibrosis patients to guide elexacaftor/ tezacaftor/ivacaftor out-of-label prescription
EUROPEAN RESPIRATORY JOURNAL
IF21
2023-09-1133
PRE
Dreano, Elise; Burgel, Pierre Regis; Hatton, Aurelie; Bouazza, Naim; Chevalier, Benoit; Macey, Julie; Leroy, Sylvie; Durieu, Isabelle; Weiss, Laurence; Grenet, Dominique; Stremler, Nathalie; Ohlmann, Camille; Reix, Philippe; Porzio, Michele; Claude, Pauline Roux; Remus, Natacha; Douvry, Benoit; Montcouquiol, Sylvie; Cosson, Laure; Mankikian, Julie; Languepin, Jeanne; Houdouin, Veronique; Le Clainche, Laurence; Guillaumot, Anne; Pouradier, Delphine; Tissot, Adrien; Priou, Pascaline; Mely, Laurent; Chedevergne, Frederique; Lebourgeois, Muriel; Lebihan, Jean; Martin, Clemence; Zavala, Flora; Da Silva, Jennifer; Lemonnier, Lydie; Kelly-Aubert, Mairead; Golec, Anita; Foucaud, Pierre; Marguet, Christophe; Edelman, Aleksander; Hinzpeter, Alexandre; de Carli, Paola; Girodon, Emmanuelle; Sermet-Gaudelus, Isabelle; Pranke, Iwona
Keratin 8 is a scaffolding and regulatory protein of ERAD complexes
CELLULAR AND MOLECULAR LIFE SCIENCES
IF6.2
2022-09-013
OA
Pranke, Iwona Maria; Chevalier, Benoit; Premchandar, Aiswarya; Baatallah, Nesrine; Tomaszewski, Kamil F.; Bitam, Sara; Tondelier, Danielle; Golec, Anita; Stolk, Jan; Lukacs, Gergely L.; Hiemstra, Pieter S.; Dadlez, Michal; Lomas, David A.; Irving, James A.; Delaunay-Moisan, Agnes; van Anken, Eelco; Hinzpeter, Alexandre; Sermet-Gaudelus, Isabelle; Edelman, Aleksander
Systemic bis-phosphinic acid derivative restores chloride transport in Cystic Fibrosis mice
SCIENTIFIC REPORTS
IF3.9
2022-04-122
OAAI
da Cunha, Melanie Faria; Pranke, Iwona; Sassi, Ali; Schreiweis, Christiane; Moriceau, Stephanie; Vidovic, Dragana; Hatton, Aurelie; Carlon, Mariane Sylvia; Creste, Geordie; Berhal, Farouk; Prestat, Guillaume; Freund, Romain; Odolczyk, Norbert; Jais, Jean Philippe; Gravier-Pelletier, Christine; Zielenkiewicz, Piotr; Jullien, Vincent; Hinzpeter, Alexandre; Oury, Franck; Edelman, Aleksander; Sermet-Gaudelus, Isabelle
Pharmacological chaperones improve intra-domain stability and inter-domain assembly via distinct binding sites to rescue misfolded CFTR

药理学伴侣通过不同的结合位点改善域内稳定性和域间组装，以挽救错误折叠的CFTR

CELLULAR AND MOLECULAR LIFE SCIENCES
IF6.2
2021-10-2938
OA
Baatallah, Nesrine; Elbahnsi, Ahmad; Mornon, Jean-Paul; Chevalier, Benoit; Pranke, Iwona; Servel, Nathalie; Zelli, Renaud; Decout, Jean-Luc; Edelman, Aleksander; Sermet-Gaudelus, Isabelle; Callebaut, Isabelle; Hinzpeter, Alexandre
New insights into structure and function of bis-phosphinic acid derivatives and implications for CFTR modulation (vol 11, 6842, 2021)
SCIENTIFIC REPORTS
IF3.9
2021-09-150
OAAI
Bitam, Sara; Elbahnsi, Ahmad; Creste, Geordie; Pranke, Iwona; Chevalier, Benoit; Berhal, Farouk; Hoffmann, Brice; Servel, Nathalie; Baatalah, Nesrine; Tondelier, Danielle; Hatton, Aurelie; Moquereau, Christelle; Faria Da Cunha, Melanie; Pastor, Alexandra; Lepissier, Agathe; Hinzpeter, Alexandre; Mornon, Jean-Paul; Prestat, Guillaume; Edelman, Aleksander; Callebaut, Isabelle; Gravier-Pelletier, Christine; Sermet-Gaudelus, Isabelle
New insights into structure and function of bis-phosphinic acid derivatives and implications for CFTR modulation
SCIENTIFIC REPORTS
IF3.9
2021-03-2510
OAAI
Bitam, Sara; Elbahnsi, Ahmad; Creste, Geordie; Pranke, Iwona; Chevalier, Benoit; Berhal, Farouk; Hoffmann, Brice; Servel, Nathalie; Tondelier, Danielle; Hatton, Aurelie; Moquereau, Christelle; Da Cunha, Melanie Faria; Pastor, Alexandra; Lepissier, Agathe; Hinzpeter, Alexandre; Mornon, Jean-Paul; Prestat, Guillaume; Edelman, Aleksander; Callebaut, Isabelle; Gravier-Pelletier, Christine; Sermet-Gaudelus, Isabelle
Author Correction: Airway surface liquid acidification initiates host defense abnormalities in Cystic Fibrosis
SCIENTIFIC REPORTS
IF3.9
2019-11-214
OAAI
Simonin, Juliette; Bille, Emmanuelle; Crambert, Gilles; Noel, Sabrina; Dreano, Elise; Edwards, Aurelie; Hatton, Aurelie; Pranke, Iwona; Villeret, Berengere; Cottart, Charles-Henry; Vrel, Jean-Patrick; Urbach, Valerie; Baatallah, Nesrine; Hinzpeter, Alexandre; Golec, Anita; Touqui, Lhousseine; Nassif, Xavier; Galietta, Luis J. V.; Planelles, Gabrielle; Sallenave, Jean-Michel; Edelman, Aleksander; Sermet-Gaudelus, Isabelle
Airway surface liquid acidification initiates host defense abnormalities in Cystic Fibrosis
SCIENTIFIC REPORTS
IF3.9
2019-04-2467
OAAI
Simonin, Juliette; Bille, Emmanuelle; Crambert, Gilles; Noel, Sabrina; Dreano, Elise; Edwards, Aurelie; Hatton, Aurelie; Pranke, Iwona; Villeret, Berengere; Cattart, Charles-Henry; Vrel, Jean-Patrick; Urbach, Valerie; Baatallah, Nesrine; Hinzpeter, Alexandre; Golec, Anita; Touqui, Lhousseine; Nassif, Xavier; Galietta, Luis J., V; Planelles, Gabrielle; Sallenave, Jean-Michel; Edelman, Aleksander; Sermet-Gaudelus, Isabelle

研究方向

暂未获取到该数据
I
Isabelle Sermet‐Gaudelus
H 指数:65论文数:530被引数:1.8万
A
Alexandre Hinzpeter
H 指数:25论文数:92被引数:1,904
A
Aleksander Edelman
H 指数:49论文数:217被引数:6,113
B
B. Chevalier
H 指数:24论文数:144被引数:2,130
A
Aurélie Hatton
H 指数:20论文数:63被引数:1,696
N
Nesrine Baatallah
H 指数:8论文数:18被引数:343
E
Elise Dréano
H 指数:12论文数:37被引数:763
A
Anita Golec
H 指数:10论文数:22被引数:573
I
Isabelle Callebaut
H 指数:73论文数:388被引数:1.9万
E
Emmanuelle Girodon
H 指数:30论文数:115被引数:3,599