@article{oai:nagasaki-u.repo.nii.ac.jp:00009559,
 author = {Ishibashi, Daisuke and Atarashi, Ryuichiro and Fuse, Takayuki and Nakagaki, Takehiro and Yamaguchi, Naohiro and Satoh, Katsuya and Honda, Kenya and Nishida, Noriyuki},
 issue = {9},
 journal = {Journal of Virology},
 month = {May},
 note = {Abnormal prion protein (PrP Sc) generated from the cellular isoform of PrP (PrP C) is assumed to be the main or sole component of the pathogen, called prion, of transmissible spongiform encephalopathies (TSE). Because PrP is a host-encoded protein, acquired immune responses are not induced in TSE. Meanwhile, activation of the innate immune system has been suggested to partially block the progression of TSE; however, the mechanism is not well understood. To further elucidate the role of the innate immune system in prion infection, we investigated the function of interferon regulatory factor 3 (IRF3), a key transcription factor of the MyD88-independent type I interferon (IFN) production pathway. We found that IRF3-deficient mice exhibited significantly earlier onset with three murine TSE strains, namely, 22L, FK-1, and murine bovine spongiform encephalopathy (mBSE), following intraperitoneal transmission, than with wild-type controls. Moreover, overexpression of IRF3 attenuated prion infection in the cell culture system, while PrP Sc was increased in prion-infected cells treated with small interfering RNAs (siRNAs) against IRF3, suggesting that IRF3 negatively regulates PrP Sc formation. Our findings provide new insight into the role of the host innate immune system in the pathogenesis of prion diseases., Journal of Virology, 86(9), pp.4947-4955; 2012},
 pages = {4947--4955},
 title = {Protective Role of Interferon Regulatory Factor 3-Mediated Signaling against Prion Infection},
 volume = {86},
 year = {2012}
}