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Reduced hnRNPA3 increases C9orf72 repeat RNA levels and dipeptide-repeat protein deposition.
Mori K, Nihei Y, Arzberger T, Zhou Q, Mackenzie IR, Hermann A, Hanisch F; German Consortium for Frontotemporal Lobar Degeneration; Bavarian Brain Banking Alliance; Kamp F, Nuscher B, Orozco D, Edbauer D, Haass C. Mori K, et al. Among authors: zhou q. EMBO Rep. 2016 Sep;17(9):1314-25. doi: 10.15252/embr.201541724. Epub 2016 Jul 26. EMBO Rep. 2016. PMID: 27461252 Free PMC article.
Antibodies inhibit transmission and aggregation of C9orf72 poly-GA dipeptide repeat proteins.
Zhou Q, Lehmer C, Michaelsen M, Mori K, Alterauge D, Baumjohann D, Schludi MH, Greiling J, Farny D, Flatley A, Feederle R, May S, Schreiber F, Arzberger T, Kuhm C, Klopstock T, Hermann A, Haass C, Edbauer D. Zhou Q, et al. EMBO Mol Med. 2017 May;9(5):687-702. doi: 10.15252/emmm.201607054. EMBO Mol Med. 2017. PMID: 28351931 Free PMC article.
Spinal poly-GA inclusions in a C9orf72 mouse model trigger motor deficits and inflammation without neuron loss.
Schludi MH, Becker L, Garrett L, Gendron TF, Zhou Q, Schreiber F, Popper B, Dimou L, Strom TM, Winkelmann J, von Thaden A, Rentzsch K, May S, Michaelsen M, Schwenk BM, Tan J, Schoser B, Dieterich M, Petrucelli L, Hölter SM, Wurst W, Fuchs H, Gailus-Durner V, de Angelis MH, Klopstock T, Arzberger T, Edbauer D. Schludi MH, et al. Among authors: zhou q. Acta Neuropathol. 2017 Aug;134(2):241-254. doi: 10.1007/s00401-017-1711-0. Epub 2017 Apr 13. Acta Neuropathol. 2017. PMID: 28409281 Free PMC article.
Cell-to-cell transmission of C9orf72 poly-(Gly-Ala) triggers key features of ALS/FTD.
Khosravi B, LaClair KD, Riemenschneider H, Zhou Q, Frottin F, Mareljic N, Czuppa M, Farny D, Hartmann H, Michaelsen M, Arzberger T, Hartl FU, Hipp MS, Edbauer D. Khosravi B, et al. Among authors: zhou q. EMBO J. 2020 Apr 15;39(8):e102811. doi: 10.15252/embj.2019102811. Epub 2020 Mar 16. EMBO J. 2020. PMID: 32175624 Free PMC article.
Drug screen in iPSC-Neurons identifies nucleoside analogs as inhibitors of (G4C2)n expression in C9orf72 ALS/FTD.
Czuppa M, Dhingra A, Zhou Q, Schludi C, König L, Scharf E, Farny D, Dalmia A, Täger J, Castillo-Lizardo M, Katona E, Mori K, Aumer T, Schelter F, Müller M, Carell T, Kalliokoski T, Messinger J, Rizzu P, Heutink P, Edbauer D. Czuppa M, et al. Among authors: zhou q. Cell Rep. 2022 Jun 7;39(10):110913. doi: 10.1016/j.celrep.2022.110913. Cell Rep. 2022. PMID: 35675776 Free article.
Targeting the glycine-rich domain of TDP-43 with antibodies prevents its aggregation in vitro and reduces neurofilament levels in vivo.
Riemenschneider H, Simonetti F, Sheth U, Katona E, Roth S, Hutten S, Farny D, Michaelsen M, Nuscher B, Schmidt MK, Flatley A, Schepers A, Gruijs da Silva LA, Zhou Q, Klopstock T, Liesz A, Arzberger T, Herms J, Feederle R, Gendron TF, Dormann D, Edbauer D. Riemenschneider H, et al. Among authors: zhou q. Acta Neuropathol Commun. 2023 Jul 11;11(1):112. doi: 10.1186/s40478-023-01592-z. Acta Neuropathol Commun. 2023. PMID: 37434215 Free PMC article.
Poly-glycine-alanine exacerbates C9orf72 repeat expansion-mediated DNA damage via sequestration of phosphorylated ATM and loss of nuclear hnRNPA3.
Nihei Y, Mori K, Werner G, Arzberger T, Zhou Q, Khosravi B, Japtok J, Hermann A, Sommacal A, Weber M; German Consortium for Frontotemporal Lobar Degeneration; Bavarian Brain Banking Alliance; Kamp F, Nuscher B, Edbauer D, Haass C. Nihei Y, et al. Among authors: zhou q. Acta Neuropathol. 2020 Jan;139(1):99-118. doi: 10.1007/s00401-019-02082-0. Epub 2019 Oct 23. Acta Neuropathol. 2020. PMID: 31642962 Free PMC article.
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