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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: shelkovnikova t. 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.
Fused in sarcoma (FUS) protein lacking nuclear localization signal (NLS) and major RNA binding motifs triggers proteinopathy and severe motor phenotype in transgenic mice.
Shelkovnikova TA, Peters OM, Deykin AV, Connor-Robson N, Robinson H, Ustyugov AA, Bachurin SO, Ermolkevich TG, Goldman IL, Sadchikova ER, Kovrazhkina EA, Skvortsova VI, Ling SC, Da Cruz S, Parone PA, Buchman VL, Ninkina NN. Shelkovnikova TA, et al. J Biol Chem. 2013 Aug 30;288(35):25266-25274. doi: 10.1074/jbc.M113.492017. Epub 2013 Jul 18. J Biol Chem. 2013. PMID: 23867462 Free PMC article.
[A mice model of amyotrophic lateral sclerosis expressing mutant human FUS protein].
Deĭkin AV, Kovrazhkina EA, Ovchinnikov RK, Bronovitskiĭ EV, Razinskaia OD, Smirnov AP, Ermolkevich TG, Eliakov AB, Popov AN, Fedorov EN, Lytkina OA, Kukharskiĭ MS, Tarasova TV, Shelkovnikova TA, Ustiugov AA, Ninkina NN, Gol'dman IL, Sadchikova ER, Bachurin SO, Skvortsova VI. Deĭkin AV, et al. Among authors: shelkovnikova ta. Zh Nevrol Psikhiatr Im S S Korsakova. 2014;114(8):62-9. Zh Nevrol Psikhiatr Im S S Korsakova. 2014. PMID: 25345633 Russian.
61 results