Lentiviral vector-mediated overexpression of mutant ataxin-7 recapitulates SCA7 pathology and promotes accumulation of the FUS/TLS and MBNL1 RNA-binding proteins

被引:0
作者
Sandro Alves
Thibaut Marais
Maria-Grazia Biferi
Denis Furling
Martina Marinello
Khalid El Hachimi
Nathalie Cartier
Merle Ruberg
Giovanni Stevanin
Alexis Brice
Martine Barkats
Annie Sittler
机构
[1] Sorbonne Universités UPMC,INSERM U 1127, CNRS UMR 7225
[2] Univ Paris 06 UMR_S 1127,CNRS FRE3617, Center for Research in Myology
[3] ICM (Brain and Spine Institute) Pitié-Salpêtrière Hospital,EPHE Ecole Pratique des Hautes Etudes, Laboratoire de Neurogénétique
[4] Sorbonne Universités UPMC Univ Paris 06,Département de Génétique et Cytogénétique
[5] INSERM UMRS974,undefined
[6] Institut de Myologie,undefined
[7] PSL Universités,undefined
[8] INSERM UMR1169,undefined
[9] MIRCen,undefined
[10] AP-HP,undefined
来源
Molecular Neurodegeneration | / 11卷
关键词
Lentiviral vector; SCA7 mouse model; Ataxia; SCA7 patients; RNA binding-proteins;
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