Auteurs
Veit G, Xu H, Dreano E, Avramescu RG, Bagdany M, Beitel LK, Roldan A, Hancock MA, Lay C, Li W, Morin K, Gao S, Mak PA, Ainscow E, Orth AP, McNamara P, Edelman A, Frenkiel S, Matouk E, Sermet-Gaudelus I, Barnes WG, Lukacs GL
Structure-guided combination therapy to potently improve the function of mutant CFTRs
Date revue
Doi
10.1038/s41591-018-0200-x
Revue
Nat Med
Auteurs
Baatallah N, Elbahnsi A, Mornon JP, Chevalier B, Pranke I, Servel N, Zelli R, Décout JL, Edelman A, Sermet-Gaudelus I, Callebaut I, Hinzpeter A
Pharmacological chaperones improve intra-domain stability and inter-domain assembly via distinct binding sites to rescue misfolded CFTR
Date revue
Doi
10.1007/s00018-021-03994-5
Revue
Cell Mol Life Sci
Auteurs
Pranke IM, Chevalier B, Premchandar A, Baatallah N, Tomaszewski KF, Bitam S, Tondelier D, Golec A, Stolk J, Lukacs GL, Hiemstra PS, Dadlez M, Lomas DA, Irving JA, Delaunay-Moisan A, van Anken E, Hinzpeter A, Sermet-Gaudelus I, Edelman A
Keratin 8 is a scaffolding and regulatory protein of ERAD complexes
Date revue
Doi
10.1007/s00018-022-04528-3
Revue
Cell Mol Life Sci
Auteurs
Hadchouel A, Drummond D, Pontoizeau C, Aoust L, Hurtado Nedelec MM, El Benna J, Gachelin E, Perisson C, Vigier C, Schiff M, Lacaille F, Molina TJ, Berteloot L, Renolleau S, Ottolenghi C, Tréluyer JM, de Blic J, Delacourt C
Methionine supplementation for multi-organ dysfunction in MetRS-related pulmonary alveolar proteinosis
Date revue
Doi
10.1183/13993003.01554-2021
Revue
Eur Respir J
Auteurs
Dreano E, Burgel PR, Hatton A, Bouazza N, Chevalier B, Macey J, Leroy S, Durieu I, Weiss L, Grenet D, Stremler N, Ohlmann C, Reix P, Porzio M, Roux Claude P, Rémus N, Douvry B, Montcouquiol S, Cosson L, Mankikian J, Languepin J, Houdouin V, Le Clainche L, Guillaumot A, Pouradier D, Tissot A, Priou P, Mély L, Chedevergne F, Lebourgeois M, Lebihan J, Martin C, Zavala F, Da Silva J, Lemonnier L, Kelly-Aubert M, Golec A, Foucaud P, Marguet C, Edelman A, Hinzpeter A, de Carli P, Girodon E, Sermet-Gaudelus I, Pranke I; French CF Reference Network study group
Theratyping cystic fibrosis patients to guide elexacaftor/tezacaftor/ivacaftor out-of-label prescription
Date revue
Doi
10.1183/13993003.00110-2023
Revue
Eur Respir J