Journal
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
Volume 22, Issue 22, Pages -Publisher
MDPI
DOI: 10.3390/ijms222212439
Keywords
prion; prion protein; prion disease; neurodegenerative disease; virus infection; conformational conversion; influenza virus; protein polymerization
Funding
- JSPS KAKENHI [19H03548, 21K07462]
- Takeda Science Foundation
- Kobayashi Magobe Memorial Medical Foundation
- Waksman Foundation of Japan
- Grants-in-Aid for Scientific Research [19H03548, 21K07462] Funding Source: KAKEN
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The article discusses the mechanism of PrP(C) conversion into PrPSc in different types of prion diseases, involving sporadic, hereditary, and acquired disorders, as well as the pathogenic role of neurotropic influenza A virus (IAV) in sporadic prion diseases.
Conformational conversion of the cellular isoform of prion protein, PrP(C), into the abnormally folded, amyloidogenic isoform, PrPSc, is an underlying pathogenic mechanism in prion diseases. The diseases manifest as sporadic, hereditary, and acquired disorders. Etiological mechanisms driving the conversion of PrP(C) into PrPSc are unknown in sporadic prion diseases, while prion infection and specific mutations in the PrP gene are known to cause the conversion of PrP(C) into PrPSc in acquired and hereditary prion diseases, respectively. We recently reported that a neurotropic strain of influenza A virus (IAV) induced the conversion of PrP(C) into PrPSc as well as formation of infectious prions in mouse neuroblastoma cells after infection, suggesting the causative role of the neuronal infection of IAV in sporadic prion diseases. Here, we discuss the conversion mechanism of PrP(C) into PrPSc in different types of prion diseases, by presenting our findings of the IAV infection-induced conversion of PrP(C) into PrPSc and by reviewing the so far reported transgenic animal models of hereditary prion diseases and the reverse genetic studies, which have revealed the structure-function relationship for PrP(C) to convert into PrPSc after prion infection.
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