Mitochondrial cardiomyopathies are fatal diseases, with no effective treatment. Alterations of
heart mitochondrial function activate the mitochondrial integrated stress response (ISR mt), a …

Mutations in CHCHD10, a mitochondrial protein with undefined functions, are associated
with autosomal dominant mitochondrial diseases. Chchd10 knock-in mice harboring a …

Mitochondrial stress triggers a response in the cell's mitochondria and nucleus, but how
these stress responses are coordinated in vivo is poorly understood. Here, we characterize …

Mitochondrial dysfunction is becoming one of the main pathology factors involved in the
etiology of neurological disorders. Recently, mutations of the coiled-coil-helix-coiled-coil …

Mutations in coiled-coil-helix–coiled-coil-helix domain containing 10 (CHCHD10), a
mitochondrial protein of unknown function, cause a disease spectrum with clinical features …

Mutations in CHCHD2 have been identified in some Parkinson's disease (PD) cases. To
understand the physiological and pathological roles of CHCHD2, we manipulated the …

Electron transport chain (ETC) defects occurring from mitochondrial disease mutations
compromise ATP synthesis and render cells vulnerable to nutrient and oxidative stress …

Mutations in CHCHD10, coding for a mitochondrial intermembrane space protein, are a rare
cause of autosomal dominant amyotrophic lateral sclerosis. Mutation-specific toxic gain of …

CHCHD2 mutations have been identified in various neurological diseases such as
Parkinson's disease (PD), frontotemporal dementia (FTD), and Alzheimer's disease (AD). It …

Rare mutations in the mitochondrial protein coiled-coil-helix-coiled-coil-helix domain
containing 2 (CHCHD2) are associated with Parkinson's disease (PD) and other Lewy body …