Mitophagy dysfunction in mitochondrial muscle disease

Mitophagy  Human Diseases

Mitophagy is a quality control mechanism that eliminates damaged mitochondria, yet its significance in mammalian pathophysiology and aging has remained unclear. Here, Mito et al., report that mitophagy contributes to mitochondrial dysfunction in skeletal muscle of aged mice and human patients.

The early disease stage is characterized by muscle fibers with central nuclei, with enhanced mitophagy around these nuclei. However, progressive mitochondrial dysfunction halts mitophagy and disrupts lysosomal homeostasis. Interestingly, activated or halted mitophagy occur in a mosaic manner even in adjacent muscle fibers, indicating cell-autonomous regulation. Rapamycin restores mitochondrial turnover, indicating mTOR-dependence of mitochondrial recycling in advanced disease stage. 

Their evidence suggests that:

  • Mitophagy is a hallmark of age-related mitochondrial pathology in mammalian muscle.
  • Mosaic halting of mitophagy is a mechanism explaining mosaic respiratory chain deficiency and accumulation of pathogenic mtDNA variants in adult-onset mitochondrial diseases and normal aging.
  • Augmenting mitophagy is a promising therapeutic approach for muscle mitochondrial dysfunction.

We will be discussing mitochondrial dysfunctions further in the 13th World Congress on Targeting Mitochondria held on October 26-28, 2022 in Berlin.

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Targeting Mitochondria 2022 Congress
October 26-28, 2022 - Berlin, Germany

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