Publications by authors named "Alexandra J Gilsrud"
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.
Mitochondria-targeted Keima (mt-Keima) is a pH-sensitive, acid-stable fluorescent protein used for the quantification of mitophagy. Mt-Keima contains a mitochondrial matrix targeting sequence and has bimodal excitation with peaks at 440 nM in neutral environments and 586 nM in acidic environments. From this bimodal excitation, a ratiometric signal may be calculated to quantify mitophagy in live cells.
View Article and Find Full Text PDFMitochondrial 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).
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