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Article

Motor-Coordinative and Cognitive Dysfunction Caused by Mutant TDP-43 Could Be Reversed by Inhibiting Its Mitochondrial Localization.

Molecular therapy : the journal of the American Society of Gene Therapy - 4 Jan 2017

Wang Wenzhang, Arakawa Hiroyuki, Wang Luwen, Okolo Ogoegbunam, Siedlak Sandra L, Jiang Yinfei, Gao Ju, Xie Fei, Petersen Robert B, Wang Xinglong

Abstract excerpt

Dominant missense mutations in TAR DNA-binding protein 43 (TDP-43) cause amyotrophic lateral sclerosis (ALS), and the cytoplasmic accumulation of TDP-43 represents a pathological hallmark in ALS and frontotemporal lobar degeneration (FTD). Behavioral investigation of the transgenic mouse model expressing the disease-causing human TDP-43 M337V mutant (TDP-43M337V mice) is encumbered by premature death in...

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PMID: 28129109
DOI: 10.1016/j.ymthe.2016.10.013
PMCID: PMC5363201

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