CORDIS Project
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This project investigates the role of mitophagy in a mouse model of COX deficiency, a mitochondrial disease. By using advanced imaging techniques and a new myoblast model, it aims to understand how mitophagy affects muscle regeneration and to identify potential drug candidates for treating mitochondrial disorders.
Mutations in COX15 are associated to cytochrome c oxidase (COX) deficiency (OMIM: 220110), a mitochondrial disease characterized by inefficient energy production due to the accumulation of impaired mitochondria.
The phenotype of a muscle-specific Cox15 knockout (Cox15sm/sm), a model of severe mitochondrial myopathy characterized by profound COX deficiency, was previously shown to be ameliorated by the coordinated induction of autophagy and lysosomal biogenesis via TFEB activation.
Stimulation of…
UNIVERSITA DEGLI STUDI DI PADOVA
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