神经损伤与功能重建2022，Vol.17Issue(9)：558,1.DOI:10.16780/j.cnki.sjssgncj.2022.09.018
小胶质细胞缺乏加速朊病毒病但不增强朊病毒在大脑中的积累
Microglia deficiency accelerates prion disease but does not enhance prion accumulation in the brain
Barry M Bradford 1Lynne I McGuire 1David A Hume 1Clare Pridans 1Neil A Mabbott 1杜一星1
作者信息
摘要
Abstract
Prion diseases are transmissible, neurodegenerative disorders associated with misfolding of the prion pro-tein. Previous studies show that reduction of microglia accelerates central nervous system (CNS) prion disease and in-creases the accumulation of prions in the brain, suggesting that microglia provide neuroprotection by phagocytosing and destroying prions. In Csf1rΔFIRE mice, the deletion of an enhancer within Csf1r specifically blocks microglia de-velopment, however, their brains develop normally and show none of the deficits reported in other microglia-defi-cient models. Csf1rΔFIRE mice were used as a refined model in which to study the impact of microglia-deficiency on CNS prion disease. Although Csf1rΔFIRE mice succumbed to CNS prion disease much earlier than wild-type mice, the accumulation of prions in their brains was reduced. Instead, astrocytes displayed earlier, non-polarized reac-tive activation with enhanced phagocytosis of neuronal contents and unfolded protein responses. Our data suggest that rather than simply phagocytosing and destroying prions, the microglia instead provide host-protection during CNS prion disease and restrict the harmful activities of reactive astrocytes.关键词
中枢神经系统/小胶质细胞/神经变性/朊病毒病/反应性星形胶质细胞Key words
central nervous system/microglia/neurodegeneration/prion disease/reactive astrocyte分类
医药卫生引用本文复制引用
Barry M Bradford,Lynne I McGuire,David A Hume,Clare Pridans,Neil A Mabbott,杜一星..小胶质细胞缺乏加速朊病毒病但不增强朊病毒在大脑中的积累[J].神经损伤与功能重建,2022,17(9):558,1.
