Griffioen Kathleen, Mattson Mark P, Okun Eitan
Department of Biology and Chemistry, Liberty University, Lynchburg, VA, 24515, USA.
Laboratory of Neurosciences, National Institute on Aging Intramural Research Program, Baltimore, MD, 21224, USA.
Heliyon. 2018 Jan 18;4(1):e00508. doi: 10.1016/j.heliyon.2018.e00508. eCollection 2018 Jan.
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.
亨廷顿舞蹈症（HD）是一种常染色体显性神经退行性疾病，其特征为进行性纹状体和皮质萎缩，与神经炎症密切相关。Toll样受体是一类天然免疫受体，是神经炎症的主要途径，对神经元可塑性和神经发育具有多效性影响。我们评估了Toll样受体2、3或4的缺陷是否会影响HD的N171-82Q小鼠模型的预期寿命。我们的数据表明，纯合的Toll样受体2和3以及杂合的Toll样受体4缺陷显著延长了HD小鼠的预期寿命。我们的数据表明，多个Toll样受体途径可能参与了HD期间的神经炎症和退行性过程。
