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Article

TDP-43 induces mitochondrial damage and activates the mitochondrial unfolded protein response

17 May 2019

Abstract excerpt

Mutations in or dys-regulation of the TDP-43 gene have been associated with TDP-43 proteinopathy, a spectrum of neurodegenerative diseases including Frontotemporal Lobar Degeneration (FTLD) and Amyotrophic Lateral Sclerosis (ALS). The underlying molecular and cellular defects, however, remain unclear. Here, we report a systematic study combining analyses of patient brain samples with cellular and animal models...

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PMID: 31100073
DOI: 10.1371/journal.pgen.1007947

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