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Mutant CHCHD10 disrupts cytochrome <i>c</i> oxidation and activates mitochondrial retrograde signaling in a model of cardiomyopathy

2025-08-18

Abstract excerpt

Mutations in CHCHD10 , a mitochondrial intermembrane space (IMS) protein implicated in proteostasis and cristae maintenance, cause multi-systemic mitochondrial disease. Heterozygous Chchd10 knock-in mice modeling the human CHCHD10 S59L variant associated with Amyotrophic Lateral Sclerosis and Frontotemporal Dementia (ALS-FTD) develop a mitochondrial cardiomyopathy driven by CHCHD10 insolubility and aggregation...

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Literature Corpus work
1a223170-9c34-5803-9b60-50b1dc5a128a
DOI
10.1101/2025.08.18.668848
Open publication

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Mutant CHCHD10 disrupts cytochrome <i>c</i> oxidation and activates mitochondrial retrograde signaling in a model of cardiomyopathyDOI 10.1101/2025.08.18.668848
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