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• W3021242589 endingPage "8509" @default.
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• W3021242589 abstract "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 protein complex at cristae junctions in patient fibroblasts bearing the CHCHD10S59L mutation, the role of CHCHD10 variants in mitofilin-associated protein complexes in brain has not been examined. Here, we utilized novel CHCHD10 transgenic mouse variants (WT, R15L, & S59L), TDP-43 transgenic mice, FTLD-TDP patient brains, and transfected cells to assess the interplay between CHCHD10 and TDP-43 on mitochondrial phenotypes. We show that CHCHD10 mutations disrupt mitochondrial OPA1-mitofilin complexes in brain, associated with impaired mitochondrial fusion and respiration. Likewise, CHCHD10 levels and OPA1-mitofilin complexes are significantly reduced in brains of FTLD-TDP patients and TDP-43 transgenic mice. In cultured cells, CHCHD10 knockdown results in OPA1-mitofilin complex disassembly, while TDP-43 overexpression also reduces CHCHD10, promotes OPA1-mitofilin complex disassembly via CHCHD10, and impairs mitochondrial fusion and respiration, phenotypes that are rescued by wild type (WT) CHCHD10. These results indicate that disruption of CHCHD10-regulated OPA1-mitofilin complex contributes to mitochondrial abnormalities in FTLD-TDP and suggest that CHCHD10 restoration could ameliorate mitochondrial dysfunction in FTLD-TDP." @default.
• W3021242589 created "2020-05-13" @default.
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• W3021242589 date "2020-05-05" @default.
• W3021242589 modified "2023-10-14" @default.
• W3021242589 title "CHCHD10‐regulated OPA1‐mitofilin complex mediates TDP‐43‐induced mitochondrial phenotypes associated with frontotemporal dementia" @default.
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• W3021242589 doi "https://doi.org/10.1096/fj.201903133rr" @default.
• W3021242589 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/7482311" @default.
• W3021242589 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/32369233" @default.
• W3021242589 hasPublicationYear "2020" @default.
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