DELE1 maintains muscle proteostasis to promote growth and survival in mitochondrial myopathy

Abstract excerpt

Mitochondrial dysfunction causes devastating disorders, including mitochondrial myopathy, but how muscle senses and adapts to mitochondrial dysfunction is not well understood. Here, we used diverse mouse models of mitochondrial myopathy to show that the signal for mitochondrial dysfunction originates within mitochondria. The mitochondrial proteins OMA1 and DELE1 sensed disruption of the inner mitochondrial...

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• Adipose Tissue and Metabolism
• Metabolism and Genetic Disorders
• Mitochondrial Function and Pathology