Interleukin-6-elicited chronic neuroinflammation may decrease survival but is not sufficient to drive disease progression in a mouse model of Leigh syndrome.

ABSTRACT:

Background

Mitochondrial diseases (MDs) are genetic disorders characterized by dysfunctions in mitochondria. Clinical data suggest that additional factors, beyond genetics, contribute to the onset and progression of this group of diseases, but these influencing factors remain largely unknown. Mounting evidence indicates that immune dysregulation or distress could play a role. Clinical observations have described the co-incidence of infection and the onset of the disease as well as the worsening of symptoms following infection. These findings highlight the complex interactions between MDs and immunity and underscore the need to better understand their underlying relationships.

Results

We used Ndufs4 KO mice, a well-established mouse model of Leigh syndrome (one of the most re

SUBMITTER: Aguilar K 

PROVIDER: S-EPMC10782699 | biostudies-literature | 2024 Jan

REPOSITORIES: biostudies-literature