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Putting bicarbonate on the spot: pharmacological insights for CFTR correction in the airway epithelium.
Zajac M, Lepissier A, Dréano E, Chevalier B, Hatton A, Kelly-Aubert M, Guidone D, Planelles G, Edelman A, Girodon E, Hinzpeter A, Crambert G, Pranke I, Galietta LJV, Sermet-Gaudelus I. Zajac M, et al. Among authors: pranke i. Front Pharmacol. 2023 Dec 11;14:1293578. doi: 10.3389/fphar.2023.1293578. eCollection 2023. Front Pharmacol. 2023. PMID: 38149052 Free PMC article.
A small molecule chaperone rescues keratin-8 mediated trafficking of misfolded podocin to correct genetic Nephrotic Syndrome.
Kuzmuk V, Pranke I, Rollason R, Butler M, Ding WY, Beesley M, Waters AM, Coward RJ, Sessions R, Tuffin J, Foster RR, Mollet G, Antignac C, Edelman A, Welsh GI, Saleem MA. Kuzmuk V, et al. Among authors: pranke i. Kidney Int. 2024 Apr;105(4):744-758. doi: 10.1016/j.kint.2023.11.006. Epub 2023 Nov 22. Kidney Int. 2024. PMID: 37995908 Free article.
Theratyping cystic fibrosis patients to guide elexacaftor/tezacaftor/ivacaftor out-of-label prescription.
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. Dreano E, et al. Among authors: pranke i. Eur Respir J. 2023 Oct 19;62(4):2300110. doi: 10.1183/13993003.00110-2023. Print 2023 Oct. Eur Respir J. 2023. PMID: 37696564
Nonsense mutations accelerate lung disease and decrease survival of cystic fibrosis children.
Orenti A, Pranke I, Faucon C, Varilh J, Hatton A, Golec A, Dehillotte C, Durieu I, Reix P, Burgel PR, Grenet D, Tasset C, Gachelin E, Perisson C, Lepissier A, Dreano E, Tondelier D, Chevalier B, Weiss L, Kiefer S, Laurans M, Chiron R, Lemonnier L, Marguet C, Jung A, Edelman A, Kerem BS, Girodon E, Taulan-Cadars M, Hinzpeter A, Kerem E, Naehrlich L, Sermet-Gaudelus I; ECFSPR Steering group. Orenti A, et al. Among authors: pranke i. J Cyst Fibros. 2023 Nov;22(6):1070-1079. doi: 10.1016/j.jcf.2023.06.005. Epub 2023 Jul 6. J Cyst Fibros. 2023. PMID: 37422433
Promitotic Action of Oenothera biennis on Senescent Human Dermal Fibroblasts.
Ceccacci S, Roger K, Metatla I, Chhuon C, Tighanimine K, Fumagalli S, De Lucia A, Pranke I, Cordier C, Monti MC, Guerrera IC. Ceccacci S, et al. Among authors: pranke i. Int J Mol Sci. 2022 Dec 2;23(23):15153. doi: 10.3390/ijms232315153. Int J Mol Sci. 2022. PMID: 36499490 Free PMC article.
The U UGA C sequence provides a favorable context to ELX-02 induced CFTR readthrough.
Pranke IM, Varilh J, Hatton A, Faucon C, Girodon E, Dreano E, Chevalier B, Karri S, Reix P, Durieu I, Bidou L, Namy O, Taulan M, Hinzpeter A, Sermet-Gaudelus I. Pranke IM, et al. J Cyst Fibros. 2023 May;22(3):560-563. doi: 10.1016/j.jcf.2022.10.010. Epub 2022 Nov 16. J Cyst Fibros. 2023. PMID: 36400713 No abstract available.
Keratin 8 is a scaffolding and regulatory protein of ERAD complexes.
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. Pranke IM, et al. Cell Mol Life Sci. 2022 Sep;79(9):503. doi: 10.1007/s00018-022-04528-3. Epub 2022 Sep 1. Cell Mol Life Sci. 2022. PMID: 36045259
36 results