Article
High fat diet ameliorates mitochondrial cardiomyopathy in CHCHD10 mutant mice.
EMBO molecular medicine - 1 Jun 2024
Southwell Nneka, Manzo Onorina, Bacman Sandra, Zhao Dazhi, Sayles Nicole M, Dash Jalia, Fujita Keigo, D'Aurelio Marilena, Di Lorenzo Annarita, Manfredi Giovanni, Kawamata Hibiki
Abstract excerpt
Mutations in CHCHD10, a mitochondrial protein with undefined functions, are associated with autosomal dominant mitochondrial diseases. Chchd10 knock-in mice harboring a heterozygous S55L mutation (equivalent to human pathogenic S59L) develop a fatal mitochondrial cardiomyopathy caused by CHCHD10 aggregation and proteotoxic mitochondrial integrated stress response (mtISR). In mutant hearts, mtISR is accompanied by...
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