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, ...

Cheng et al. identify a mitochondrial complex IV (CIV) deficiency in the brains of patients with sporadic amyotrophic lateral sclerosis (ALS). They demonstrate that defects in mitochondrial CIV induce...

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Combined expression of a mitochondrially targeted endonuclease and end-joining machinery enables formation of mtDNA deletions with a broad range of heteroplasmy.

Combined expression of a mitochondrially targeted endonuclease and end-joining machinery enables formation of mtDNA deletions with a broad range of heteroplasmy.

We find that, in mice, although the individual loss of Parkin or OMA1 does not affect mitochondrial integrity, their combined loss results in small body size, low locomotor activity, premature death, ...

Organelle network is regulated to prevent cellular inflammation.

Organelle network is regulated to prevent cellular inflammation.

Mitochondrial DNA (mtDNA) copy number regulation remains incompletely understood, despite its importance in cellular function. In Saccharomyces cerevisiae, Mrx6 belongs to the Pet20-domain-containing ...

Targeting mitochondrial metabolic activity is an active area of cancer research. A study now finds that a selective deficiency in mitochondrial complex I in melanoma cells increases mitochondrial acet...

Mitochondria play central roles in metabolism and metabolic disorders such as type 2 diabetes. MIC26, a mitochondrial contact site and cristae organising system complex subunit, was linked to diabetes...