A robust evaluation of TDP-43, poly GP, cellular pathology and behavior in an AAV-C9ORF72 (G4C2)66 mouse model

A robust evaluation of TDP-43, poly GP, cellular pathology and behavior in an AAV-C9ORF72 (G4C2)66 mouse model

Abstract The G4C2 hexanucleotide repeat expansion in C9ORF72 is the major genetic cause of both amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) (C9-ALS/FTD). Despite considerable efforts, the development of mouse models of C9-ALS/FTD useful for therapeutic development has prove...

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Main Authors: Emily G. Thompson, Olivia Spead, Suleyman C. Akerman, Carrie Curcio, Benjamin L. Zaepfel, Erica R. Kent, Thomas Philips, Balaji G. Vijayakumar, Anna Zacco, Weibo Zhou, Guhan Nagappan, Jeffrey D. Rothstein
Format: Article
Language:English
Published: BMC 2024-12-01
Series:Acta Neuropathologica Communications
Subjects:
Amyotrophic lateral sclerosis
C9orf72 repeat expansion
Dipeptide repeats
P62
NfL
GFAP
Online Access:https://doi.org/10.1186/s40478-024-01911-y
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https://doi.org/10.1186/s40478-024-01911-y

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