Publications by authors named "Nicholas P Randolph"
Article Synopsis
- Mitochondrial dysfunction can lead to serious health issues like mitochondrial myopathy, but scientists aren't sure how muscles react to this problem.
- Researchers studied mice with mitochondrial myopathy and found that signals about the dysfunction come from within the mitochondria themselves.
- They discovered that specific mitochondrial proteins activated a response (called mt-ISR) that helps muscles grow and survive by fixing issues in protein production, showing that this response is important across different types of tissues.
Mitochondrial dysfunction causes devastating disorders, including mitochondrial myopathy. Here, we identified that diverse mitochondrial myopathy models elicit a protective mitochondrial integrated stress response (mt-ISR), mediated by OMA1-DELE1 signaling. The response was similar following disruptions in mtDNA maintenance, from knockout of , and mitochondrial protein unfolding, from disease-causing mutations in CHCHD10 (G58R and S59L).
View Article and Find Full Text PDFMitochondrial stress triggers a response in the cell's mitochondria and nucleus, but how these stress responses are coordinated in vivo is poorly understood. Here, we characterize a family with myopathy caused by a dominant p.G58R mutation in the mitochondrial protein CHCHD10.
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