Virus Infection, Genetic Mutations, and Prion Infection in Prion Protein Conversion

Conformational conversion of the cellular isoform of prion protein, PrP<sup>C</sup>, into the abnormally folded, amyloidogenic isoform, PrP<sup>Sc</sup>, is an underlying pathogenic mechanism in prion diseases. The diseases manifest as sporadic, hereditary, and acquired disor...

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Autores principales:	Hideyuki Hara, Suehiro Sakaguchi
Formato:	article
Lenguaje:	EN
Publicado:	MDPI AG 2021
Materias:	prion prion protein prion disease neurodegenerative disease virus infection conformational conversion Biology (General) QH301-705.5 Chemistry QD1-999
Acceso en línea:	https://doaj.org/article/21c6fb74577f46fdba3cfa2a3a51263b
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spelling	oai:doaj.org-article:21c6fb74577f46fdba3cfa2a3a51263b2021-11-25T17:56:40ZVirus Infection, Genetic Mutations, and Prion Infection in Prion Protein Conversion10.3390/ijms2222124391422-00671661-6596https://doaj.org/article/21c6fb74577f46fdba3cfa2a3a51263b2021-11-01T00:00:00Zhttps://www.mdpi.com/1422-0067/22/22/12439https://doaj.org/toc/1661-6596https://doaj.org/toc/1422-0067Conformational conversion of the cellular isoform of prion protein, PrP<sup>C</sup>, into the abnormally folded, amyloidogenic isoform, PrP<sup>Sc</sup>, is an underlying pathogenic mechanism in prion diseases. The diseases manifest as sporadic, hereditary, and acquired disorders. Etiological mechanisms driving the conversion of PrP<sup>C</sup> into PrP<sup>Sc</sup> are unknown in sporadic prion diseases, while prion infection and specific mutations in the PrP gene are known to cause the conversion of PrP<sup>C</sup> into PrP<sup>Sc</sup> in acquired and hereditary prion diseases, respectively. We recently reported that a neurotropic strain of influenza A virus (IAV) induced the conversion of PrP<sup>C</sup> into PrP<sup>Sc</sup> 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<sup>C</sup> into PrP<sup>Sc</sup> in different types of prion diseases, by presenting our findings of the IAV infection-induced conversion of PrP<sup>C</sup> into PrP<sup>Sc</sup> 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<sup>C</sup> to convert into PrP<sup>Sc</sup> after prion infection.Hideyuki HaraSuehiro SakaguchiMDPI AGarticleprionprion proteinprion diseaseneurodegenerative diseasevirus infectionconformational conversionBiology (General)QH301-705.5ChemistryQD1-999ENInternational Journal of Molecular Sciences, Vol 22, Iss 12439, p 12439 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	prion prion protein prion disease neurodegenerative disease virus infection conformational conversion Biology (General) QH301-705.5 Chemistry QD1-999
spellingShingle	prion prion protein prion disease neurodegenerative disease virus infection conformational conversion Biology (General) QH301-705.5 Chemistry QD1-999 Hideyuki Hara Suehiro Sakaguchi Virus Infection, Genetic Mutations, and Prion Infection in Prion Protein Conversion
description	Conformational conversion of the cellular isoform of prion protein, PrP<sup>C</sup>, into the abnormally folded, amyloidogenic isoform, PrP<sup>Sc</sup>, is an underlying pathogenic mechanism in prion diseases. The diseases manifest as sporadic, hereditary, and acquired disorders. Etiological mechanisms driving the conversion of PrP<sup>C</sup> into PrP<sup>Sc</sup> are unknown in sporadic prion diseases, while prion infection and specific mutations in the PrP gene are known to cause the conversion of PrP<sup>C</sup> into PrP<sup>Sc</sup> in acquired and hereditary prion diseases, respectively. We recently reported that a neurotropic strain of influenza A virus (IAV) induced the conversion of PrP<sup>C</sup> into PrP<sup>Sc</sup> 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<sup>C</sup> into PrP<sup>Sc</sup> in different types of prion diseases, by presenting our findings of the IAV infection-induced conversion of PrP<sup>C</sup> into PrP<sup>Sc</sup> 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<sup>C</sup> to convert into PrP<sup>Sc</sup> after prion infection.
format	article
author	Hideyuki Hara Suehiro Sakaguchi
author_facet	Hideyuki Hara Suehiro Sakaguchi
author_sort	Hideyuki Hara
title	Virus Infection, Genetic Mutations, and Prion Infection in Prion Protein Conversion
title_short	Virus Infection, Genetic Mutations, and Prion Infection in Prion Protein Conversion
title_full	Virus Infection, Genetic Mutations, and Prion Infection in Prion Protein Conversion
title_fullStr	Virus Infection, Genetic Mutations, and Prion Infection in Prion Protein Conversion
title_full_unstemmed	Virus Infection, Genetic Mutations, and Prion Infection in Prion Protein Conversion
title_sort	virus infection, genetic mutations, and prion infection in prion protein conversion
publisher	MDPI AG
publishDate	2021
url	https://doaj.org/article/21c6fb74577f46fdba3cfa2a3a51263b
work_keys_str_mv	AT hideyukihara virusinfectiongeneticmutationsandprioninfectioninprionproteinconversion AT suehirosakaguchi virusinfectiongeneticmutationsandprioninfectioninprionproteinconversion
_version_	1718411789055557632

Virus Infection, Genetic Mutations, and Prion Infection in Prion Protein Conversion

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