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Content archived on 2024-06-18

Mitochondrial deficiency and cardiomyopathy. Role of Reactive Oxygen Species

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Mitochondria, reactive oxygen species, and cardiomyopathy

Mitochondrial diseases (MD) are among the most common forms of genetic disorders. A European project investigated the role of mitochondrial dysfunction in cardiomyopathy.

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One of the most studied clinical presentations of MD is neuromuscular dysfunction, followed by cardiomyopathy. The disease can be caused by mutations in either the nuclear or the mitochondrial DNA. Mitochondria are involved in the initiation and progression of MD through production of the reactive oxygen species (ROS). Some of the cardiomyopathy-associated mutations clearly implicate ROS and mitochondrial deficiency as an initial step in the onset of cardiomyopathy. The aim of the EU-funded ROLROS (Mitochondrial deficiency and cardiomyopathy. Role of reactive oxygen species) project was to study the involvement of mitochondria in cardiomyopathy using different MD models. During the project, researchers demonstrated that ROS is a critical factor in the different scenarios leading to MD. It affected the in vitro differentiation of mouse embryonic stem cells into cardiomyocytes as well as metabolic adaptation in neonatal cardiomyocytes. In in vivo experimental models of heart failure, ROS acted as an initiator of a signalling cascade that ultimately led to cardiomyocyte death. Based on the established proof of principle experiments, researchers were able to identify genotypes that were more susceptible to cardiac damage. The data obtained make it possible to predict novel putative target proteins for the treatment of MD-associated cardiomyopathy.

Keywords

Mitochondria, reactive oxygen species, cardiomyopathy, ROLROS, cardiomyocytes

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