Article
Loss of mitochondrial Chchd10 or Chchd2 in zebrafish leads to an ALS-like phenotype and Complex I deficiency independent of the mitochondrial integrated stress response.
Developmental neurobiology - 1 Jan 2023
Petel Légaré Virginie, Rampal Christian J, Gurberg Tyler J N, Aaltonen Mari J, Janer Alexandre, Zinman Lorne, Shoubridge Eric A, Armstrong Gary A B
Abstract excerpt
Mutations in CHCHD10 and CHCHD2, encoding two paralogous mitochondrial proteins, have been identified in cases of amyotrophic lateral sclerosis, frontotemporal lobar degeneration, and Parkinson's disease. Their role in disease is unclear, though both have been linked to mitochondrial respiration and mitochondrial stress responses. Here, we investigated the biological roles of these proteins during vertebrate...
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