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Early molecular layer interneuron hyperactivity triggers Purkinje neuron degeneration in SCA1.
Pilotto F, Douthwaite C, Diab R, Ye X, Al Qassab Z, Tietje C, Mounassir M, Odriozola A, Thapa A, Buijsen RAM, Lagache S, Uldry AC, Heller M, Müller S, van Roon-Mom WMC, Zuber B, Liebscher S, Saxena S. Pilotto F, et al. Among authors: diab r. Neuron. 2023 Aug 16;111(16):2523-2543.e10. doi: 10.1016/j.neuron.2023.05.016. Epub 2023 Jun 14. Neuron. 2023. PMID: 37321222 Free PMC article.
PolyGA targets the ER stress-adaptive response by impairing GRP75 function at the MAM in C9ORF72-ALS/FTD.
Pilotto F, Schmitz A, Maharjan N, Diab R, Odriozola A, Tripathi P, Yamoah A, Scheidegger O, Oestmann A, Dennys CN, Sinha Ray S, Rodrigo R, Kolb S, Aronica E, Di Santo S, Widmer HR, Charlet-Berguerand N, Selvaraj BT, Chandran S, Meyer K, Zuber B, Goswami A, Weis J, Saxena S. Pilotto F, et al. Among authors: diab r. Acta Neuropathol. 2022 Nov;144(5):939-966. doi: 10.1007/s00401-022-02494-5. Epub 2022 Sep 19. Acta Neuropathol. 2022. PMID: 36121477 Free PMC article.