Unknown

Dataset Information

0

Murine cytomegalovirus infection exacerbates complex IV deficiency in a model of mitochondrial disease.


ABSTRACT: 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.

SUBMITTER: Ferreira N 

PROVIDER: S-EPMC7055822 | biostudies-literature | 2020 Mar

REPOSITORIES: biostudies-literature

altmetric image

Publications


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 infec  ...[more]

Similar Datasets

| S-EPMC7247164 | biostudies-literature
| S-EPMC5428769 | biostudies-literature
| S-EPMC4654296 | biostudies-other
2020-02-18 | E-MTAB-7503 | biostudies-arrayexpress
| S-EPMC6670028 | biostudies-literature
| S-EPMC6078326 | biostudies-literature
| S-EPMC8753920 | biostudies-literature
| S-EPMC5154339 | biostudies-literature
| S-EPMC2947306 | biostudies-literature
| S-EPMC4297913 | biostudies-literature