The role of mtDNA integrity in muscle progenitor function remains poorly understood. Sen et al. show that mtDNA alterations in myoblasts impair myogenic differentiation and reshape the mitochondrial p...

Zhang et al. characterize the mitochondrial inner membrane subdomain organizer (MISO) protein, which responds to inner mitochondrial membrane stress by inducing membrane subdomains promoting homeostat...

An activator of DNA polymerase γ restores function to disease-causing mutant variants and demonstrates a potential route to treatments for inherited mitochondrial disorders involving POLG mutations.

Damage to the mitochondria, the “power plants” of the cells, contributes to many diseases. Researchers from Heinrich Heine University Düsseldorf (HHU) and the University of Cologne led by HHU professo...

Lysosomal uptake of mtDNA controls mitochondrial quality.

Lysosomal uptake of mtDNA controls mitochondrial quality.

Pathogenic variants in the mitochondrial protein MFN2 are typically associated with a peripheral neuropathy phenotype, but can also cause a variety of additional pathologies including myopathy. Here, ...