Both monogenic diseases and viral infections can manifest in a broad spectrum of clinical phenotypes that range from asymptomatic to lethal, suggesting that other factors modulate disease severity. Here, we examine the interplay between the genetic neuronopathic Gaucher’s disease (nGD), and neuroinvasive Sindbis virus (SVNI) infection. Infection of nGD mice with SVNI had no influence on nGD severity. However, nGD mice were more resistant to SVNI infection. Significantly different inflammatory responses were seen in nGD brains when compared with SVNI brains: the inflammatory response in the nGD brains consisted of reactive astrocytes and microglia with no infiltrating macrophages, but the inflammatory response in the brains of SVNI-infected mice was characterized by infiltration of macrophages and altered activation of microglia and astrocytes. We suggest that the innate immune response activated in nGD confers resistance against viral infection of the CNS.

中文翻译：

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神经病性高雪氏病的先天免疫应答赋予抵抗病毒性脑炎的抵抗力。

单基因疾病和病毒感染均可在从无症状到致死的广泛临床表型中体现，表明其他因素可调节疾病的严重程度。在这里，我们检查了遗传神经病性高雪氏病（nGD）与神经浸润性辛德比斯病毒（SVNI）感染之间的相互作用。SVNI感染nGD小鼠对nGD严重性没有影响。但是，nGD小鼠对SVNI感染的抵抗力更高。与SVNI大脑相比，在nGD大脑中观察到了明显不同的炎症反应：nGD大脑中的炎症反应由反应性星形胶质细胞和小胶质细胞组成，没有浸润性巨噬细胞，但是SVNI感染小鼠的大脑中的炎症反应的特征在于巨噬细胞和小胶质细胞和星形胶质细胞活化的改变。