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Our unpublished findings indicate that genetic ablation of the mitochondrial mRNA-stabilizing protein, LRPPRC, leads to mitochondrial myopathy and nerve-muscular-junction (NMJ) instability, demonstrating the link between muscle mitochondria and NMJ regulation. Our research also shows that exercise elevates the content of muscle LRPPRC protein and increases overall mitochondrial function, suggesting exercise as a potential therapeutic intervention for mitochondrial myopathies.
Our project comprises two work packages: the first will determine the molecular muscle dysregulations in patients with mitochondrial myopathy that are reversible by exercise. The second work package will evaluate drugs, informed by the first work package´s findings, for their potential to restore muscle function in preclinical models of mitochondrial myopathy.
Ultimately, these insights into mitochondrial myopathy could help decipher muscle-nerve communication and lead to improved treatments that could enhance patient quality of life and reduce healthcare costs.