| First Author | Ferreira N | Year | 2020 |
| Journal | PLoS Genet | Volume | 16 |
| Issue | 3 | Pages | e1008604 |
| PubMed ID | 32130224 | Mgi Jnum | J:285887 |
| Mgi Id | MGI:6400015 | Doi | 10.1371/journal.pgen.1008604 |
| Citation | Ferreira N, et al. (2020) Murine cytomegalovirus infection exacerbates complex IV deficiency in a model of mitochondrial disease. PLoS Genet 16(3):e1008604 |
| abstractText | The influence of environmental insults on the onset and progression of mitochondrial diseases is unknown. To evaluate the effects of infection on mitochondrial disease we used a mouse model of Leigh Syndrome, where a missense mutation in the Taco1 gene results in the loss of the translation activator of cytochrome c oxidase subunit I (TACO1) protein. The mutation leads to an isolated complex IV deficiency that mimics the disease pathology observed in human patients with TACO1 mutations. We infected Taco1 mutant and wild-type mice with a murine cytomegalovirus and show that a common viral infection exacerbates the complex IV deficiency in a tissue-specific manner. We identified changes in neuromuscular morphology and tissue-specific regulation of the mammalian target of rapamycin pathway in response to viral infection. Taken together, we report for the first time that a common stress condition, such as viral infection, can exacerbate mitochondrial dysfunction in a genetic model of mitochondrial disease. |
