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Atxn2-CAG100-KnockIn mouse spinal cord shows progressive TDP43 pathology associated with cholesterol biosynthesis suppression.
Canet-Pons J, Sen NE, Arsović A, Almaguer-Mederos LE, Halbach MV, Key J, Döring C, Kerksiek A, Picchiarelli G, Cassel R, René F, Dieterlé S, Fuchs NV, König R, Dupuis L, Lütjohann D, Gispert S, Auburger G. Canet-Pons J, et al. Among authors: dieterle s. Neurobiol Dis. 2021 May;152:105289. doi: 10.1016/j.nbd.2021.105289. Epub 2021 Feb 10. Neurobiol Dis. 2021. PMID: 33577922 Free article.
Serotonin 2B receptor slows disease progression and prevents degeneration of spinal cord mononuclear phagocytes in amyotrophic lateral sclerosis.
El Oussini H, Bayer H, Scekic-Zahirovic J, Vercruysse P, Sinniger J, Dirrig-Grosch S, Dieterlé S, Echaniz-Laguna A, Larmet Y, Müller K, Weishaupt JH, Thal DR, van Rheenen W, van Eijk K, Lawson R, Monassier L, Maroteaux L, Roumier A, Wong PC, van den Berg LH, Ludolph AC, Veldink JH, Witting A, Dupuis L. El Oussini H, et al. Among authors: dieterle s. Acta Neuropathol. 2016 Mar;131(3):465-80. doi: 10.1007/s00401-016-1534-4. Epub 2016 Jan 7. Acta Neuropathol. 2016. PMID: 26744351 Free article.
Toxic gain of function from mutant FUS protein is crucial to trigger cell autonomous motor neuron loss.
Scekic-Zahirovic J, Sendscheid O, El Oussini H, Jambeau M, Sun Y, Mersmann S, Wagner M, Dieterlé S, Sinniger J, Dirrig-Grosch S, Drenner K, Birling MC, Qiu J, Zhou Y, Li H, Fu XD, Rouaux C, Shelkovnikova T, Witting A, Ludolph AC, Kiefer F, Storkebaum E, Lagier-Tourenne C, Dupuis L. Scekic-Zahirovic J, et al. Among authors: dieterle s. EMBO J. 2016 May 17;35(10):1077-97. doi: 10.15252/embj.201592559. Epub 2016 Mar 7. EMBO J. 2016. PMID: 26951610 Free PMC article.
Motor neuron intrinsic and extrinsic mechanisms contribute to the pathogenesis of FUS-associated amyotrophic lateral sclerosis.
Scekic-Zahirovic J, Oussini HE, Mersmann S, Drenner K, Wagner M, Sun Y, Allmeroth K, Dieterlé S, Sinniger J, Dirrig-Grosch S, René F, Dormann D, Haass C, Ludolph AC, Lagier-Tourenne C, Storkebaum E, Dupuis L. Scekic-Zahirovic J, et al. Among authors: dieterle s. Acta Neuropathol. 2017 Jun;133(6):887-906. doi: 10.1007/s00401-017-1687-9. Epub 2017 Feb 28. Acta Neuropathol. 2017. PMID: 28243725 Free PMC article.
FUS-mediated regulation of acetylcholine receptor transcription at neuromuscular junctions is compromised in amyotrophic lateral sclerosis.
Picchiarelli G, Demestre M, Zuko A, Been M, Higelin J, Dieterlé S, Goy MA, Mallik M, Sellier C, Scekic-Zahirovic J, Zhang L, Rosenbohm A, Sijlmans C, Aly A, Mersmann S, Sanjuan-Ruiz I, Hübers A, Messaddeq N, Wagner M, van Bakel N, Boutillier AL, Ludolph A, Lagier-Tourenne C, Boeckers TM, Dupuis L, Storkebaum E. Picchiarelli G, et al. Among authors: dieterle s. Nat Neurosci. 2019 Nov;22(11):1793-1805. doi: 10.1038/s41593-019-0498-9. Epub 2019 Oct 7. Nat Neurosci. 2019. PMID: 31591561 Free PMC article.
Cytoplasmic FUS triggers early behavioral alterations linked to cortical neuronal hyperactivity and inhibitory synaptic defects.
Scekic-Zahirovic J, Sanjuan-Ruiz I, Kan V, Megat S, De Rossi P, Dieterlé S, Cassel R, Jamet M, Kessler P, Wiesner D, Tzeplaeff L, Demais V, Sahadevan S, Hembach KM, Muller HP, Picchiarelli G, Mishra N, Antonucci S, Dirrig-Grosch S, Kassubek J, Rasche V, Ludolph A, Boutillier AL, Roselli F, Polymenidou M, Lagier-Tourenne C, Liebscher S, Dupuis L. Scekic-Zahirovic J, et al. Among authors: dieterle s. Nat Commun. 2021 May 21;12(1):3028. doi: 10.1038/s41467-021-23187-9. Nat Commun. 2021. PMID: 34021132 Free PMC article.
Wild-type FUS corrects ALS-like disease induced by cytoplasmic mutant FUS through autoregulation.
Sanjuan-Ruiz I, Govea-Perez N, McAlonis-Downes M, Dieterle S, Megat S, Dirrig-Grosch S, Picchiarelli G, Piol D, Zhu Q, Myers B, Lee CZ, Cleveland DW, Lagier-Tourenne C, Da Cruz S, Dupuis L. Sanjuan-Ruiz I, et al. Among authors: dieterle s. Mol Neurodegener. 2021 Sep 6;16(1):61. doi: 10.1186/s13024-021-00477-w. Mol Neurodegener. 2021. PMID: 34488813 Free PMC article.
Mutant FUS induces chromatin reorganization in the hippocampus and alters memory processes.
Tzeplaeff L, Seguin J, Le Gras S, Megat S, Cosquer B, Plassard D, Dieterlé S, Paiva I, Picchiarelli G, Decraene C, Alcala-Vida R, Cassel JC, Merienne K, Dupuis L, Boutillier AL. Tzeplaeff L, et al. Among authors: dieterle s. Prog Neurobiol. 2023 Aug;227:102483. doi: 10.1016/j.pneurobio.2023.102483. Epub 2023 Jun 15. Prog Neurobiol. 2023. PMID: 37327984
51 results