DELE1 Maintains Muscle Proteostasis to Promote Growth and Survival in Mitochondrial Myopathy

Investigators 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 membrane and, in response, activated the mitochondrial integrated stress response to increase the building blocks for protein synthesis.