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Article

CHCHD10-regulated OPA1-mitofilin complex mediates TDP-43-induced mitochondrial phenotypes associated with frontotemporal dementia.

FASEB journal : official publication of the Federation of American Societies for Experimental Biology - 1 Jun 2020

Liu Tian, Woo Jung-A A, Bukhari Mohammed Zaheen, LePochat Patrick, Chacko Ann, Selenica Maj-Linda B, Yan Yan, Kotsiviras Peter, Buosi Sara Cazzaro, Zhao Xingyu, Kang David E

Abstract excerpt

Mutations in CHCHD10, a gene coding for a mitochondrial protein, are implicated in ALS-FTD spectrum disorders, which are pathologically characterized by transactive response DNA binding protein 43 kDa (TDP-43) accumulation. While both TDP-43 and CHCHD10 mutations drive mitochondrial pathogenesis, mechanisms underlying such phenotypes are unclear. Moreover, despite the disruption of the mitochondrial mitofilin...

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Identifiers

PMID: 32369233
DOI: 10.1096/fj.201903133RR
PMCID: PMC7482311

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