Deficiency of Toll-like receptors 2, 3 or 4 extends life expectancy in Huntington’s disease mice

Huntington’s disease (HD), an autosomal dominant neurodegenerative disorder characterized by progressive striatal and cortical atrophy, has been strongly linked with neuroinflammation. Toll-like receptors, a family of innate immune receptors, are a major pathway for neuroinflammation with pleiotropi...

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Bibliographic Details
Published in:Heliyon 2018-01, Vol.4 (1), p.e00508-e00508, Article e00508
Main Authors: Griffioen, Kathleen, Mattson, Mark P., Okun, Eitan
Format: Article
Language:English
Subjects:
Neurology
Neuroscience
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Summary:Huntington’s disease (HD), an autosomal dominant neurodegenerative disorder characterized by progressive striatal and cortical atrophy, has been strongly linked with neuroinflammation. Toll-like receptors, a family of innate immune receptors, are a major pathway for neuroinflammation with pleiotropic effects on neuronal plasticity and neurodevelopment. We assessed whether deficiency for TLRs 2, 3 or 4 affects life expectancy in the N171-82Q mouse model of HD. Our data indicate that homozygous TLRs 2 and 3 as well as heterozygous TLR4 deficiency significantly extends the life expectancy of HD mice. Our data suggest that multiple TLR pathways may be involved in the neuroinflammatory and degenerative processes during HD.
ISSN:2405-8440
2405-8440
DOI:10.1016/j.heliyon.2018.e00508