@article{b11c03ce249b4c7f87d2fb14753f8f71,
title = "Innate immune response in neuronopathic forms of Gaucher disease confers resistance against viral-induced encephalitis",
abstract = "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.",
author = "Sharon Melamed and Roy Avraham and Rothbard, {Deborah E} and Noam Erez and Tomer Israely and Ziv Klausner and Futerman, {Anthony H} and Nir Paran and Vitner, {Einat B}",
note = "We thank Dr. Shlomit Gilad and Dr. Sima Benjamin (The Nancy and Stephen Grand Israel National Center for Personalized Medicine, WIS) for RNA–seq and bioinformatics analysis, Dr. Raya Eilam and Amit Gross (WIS) for help with Immunofluorescence staining, Vladimir Kiss and Dr. Reinat Nevo (WIS) for help with fluorescence microscopy, Dr. Inbar Gihon (IIBR) and Dr. Ester Feldmesser (WIS) for help with RNA-seq data analysis, Muriel Chemla (Life Sciences Core Facilities, WIS) for determining RIN values and Dr. Stefan Karlsson for providing the Gba flox/flox; nestin-Cre nGD mice. R.A. is supported by the Israel Science Foundation (Grant 521/18).E.B.V is supported by the Katzir Foundation. A.H. Futerman is The Joseph Meyerhoff Professor of Biochemistry at the Weizmann Institute of Science. N.P., A.H.F., and E.B.V. designed the research; S.M., R.A., D.E.R., N.E., T.I., and E.B.V. performed the experiments; Z.K performed the statistical analysis, D.E.R., N.E., and E.B.V. analyzed data. E.B.V. wrote the manuscript. All authors discussed results and commented on the manuscript before submission. All authors read and approved the final manuscript.",
year = "2020",
month = aug,
day = "24",
doi = "10.1186/s40478-020-01020-6",
language = "الإنجليزيّة",
volume = "8",
journal = "Acta Neuropathologica Communications",
issn = "2051-5960",
publisher = "BioMed Central Ltd.",
}