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Pharmacological chaperones improve intra-domain stability and inter-domain assembly via distinct binding sites to rescue misfolded CFTR.
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. Baatallah N, et al. Among authors: edelman a. Cell Mol Life Sci. 2021 Dec;78(23):7813-7829. doi: 10.1007/s00018-021-03994-5. Epub 2021 Oct 29. Cell Mol Life Sci. 2021. PMID: 34714360 Free PMC article.
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. Among authors: edelman a. 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
Disruption of cytokeratin-8 interaction with F508del-CFTR corrects its functional defect.
Colas J, Faure G, Saussereau E, Trudel S, Rabeh WM, Bitam S, Guerrera IC, Fritsch J, Sermet-Gaudelus I, Davezac N, Brouillard F, Lukacs GL, Herrmann H, Ollero M, Edelman A. Colas J, et al. Among authors: edelman a. Hum Mol Genet. 2012 Feb 1;21(3):623-34. doi: 10.1093/hmg/ddr496. Epub 2011 Oct 28. Hum Mol Genet. 2012. PMID: 22038833 Free PMC article.
Correction of CFTR function in nasal epithelial cells from cystic fibrosis patients predicts improvement of respiratory function by CFTR modulators.
Pranke IM, Hatton A, Simonin J, Jais JP, Le Pimpec-Barthes F, Carsin A, Bonnette P, Fayon M, Stremler-Le Bel N, Grenet D, Thumerel M, Mazenq J, Urbach V, Mesbahi M, Girodon-Boulandet E, Hinzpeter A, Edelman A, Sermet-Gaudelus I. Pranke IM, et al. Among authors: edelman a. Sci Rep. 2017 Aug 7;7(1):7375. doi: 10.1038/s41598-017-07504-1. Sci Rep. 2017. PMID: 28785019 Free PMC article.
Might Brushed Nasal Cells Be a Surrogate for CFTR Modulator Clinical Response?
Pranke I, Hatton A, Masson A, Flament T, Le Bourgeois M, Chedevergne F, Bailly C, Urbach V, Hinzpeter A, Edelman A, Sermet-Gaudelus I. Pranke I, et al. Among authors: edelman a. Am J Respir Crit Care Med. 2019 Jan 1;199(1):123-126. doi: 10.1164/rccm.201808-1436LE. Am J Respir Crit Care Med. 2019. PMID: 30326728 No abstract available.
502 results