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Transmissibility of Gerstmann–Sträussler–Scheinker syndrome in rodent models: New insights into the molecular underpinnings of prion infectivity

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Pages 421-433 | Received 10 Aug 2016, Accepted 19 Sep 2016, Published online: 28 Nov 2016

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Vitaly V. Kushnirov, Alexander A. Dergalev & Alexander I. Alexandrov. (2020) Proteinase K resistant cores of prions and amyloids. Prion 14:1, pages 11-19.
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Jing Wang, Kang Xiao, Wei Zhou, Chen Gao, Cao Chen, Qi Shi & Xiao-Ping Dong. (2018) A Chinese patient of P102L Gerstmann-Sträussler-Scheinker disease contains three other disease-associated mutations in SYNE1. Prion 12:2, pages 150-155.
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Lin Chen, Yin Xu, Ming-juan Fang, Yong-guang Shi, Jie Zhang, Liang-liang Zhang, Yu Wang, Yong-zhu Han, Ji-yuan Hu, Ren-min Yang & Xu-en Yu. (2023) Case report: A Chinese patient with spinocerebellar ataxia finally confirmed as Gerstmann-Sträussler-Scheinker syndrome with P102L mutation. Frontiers in Neurology 14.
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Rosalia Bruno, Geraldina Riccardi, Floriana Iacobone, Flavia Chiarotti, Laura Pirisinu, Ilaria Vanni, Stefano Marcon, Claudia D’Agostino, Matteo Giovannelli, Piero Parchi, Umberto Agrimi, Romolo Nonno & Michele Angelo Di Bari. (2023) Strain-Dependent Morphology of Reactive Astrocytes in Human- and Animal-Vole-Adapted Prions. Biomolecules 13:5, pages 757.
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Tvisha Joshi & Nidhi Ahuja. (2023) The Prion Basis of Progressive Neurodegenerative Disorders. Interdisciplinary Perspectives on Infectious Diseases 2023, pages 1-5.
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Laura Pirisinu, Michele Angelo Di Bari, Claudia D’Agostino, Ilaria Vanni, Geraldina Riccardi, Stefano Marcon, Gabriele Vaccari, Barbara Chiappini, Sylvie L. Benestad, Umberto Agrimi & Romolo Nonno. (2022) A single amino acid residue in bank vole prion protein drives permissiveness to Nor98/atypical scrapie and the emergence of multiple strain variants. PLOS Pathogens 18:6, pages e1010646.
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Jean-Numa Gillet. (2022) From molecular dynamics to quantum mechanics of misfolded proteins and amyloid-like macroaggregates applied to neurodegenerative diseases. Journal of Molecular Graphics and Modelling 110, pages 108046.
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Melanie Alpaugh & Francesca Cicchetti. (2021) Huntington’s disease: lessons from prion disorders. Journal of Neurology 268:9, pages 3493-3504.
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Surachai Supattapone. 2020. Prions and Neurodegenerative Diseases. Prions and Neurodegenerative Diseases 53 75 .
Cassandra M. Burke, Daniel J. Walsh, Alexander D. Steele, Umberto Agrimi, Michele Angelo Di Bari, Joel C. Watts & Surachai Supattapone. (2019) Full restoration of specific infectivity and strain properties from pure mammalian prion protein. PLOS Pathogens 15:3, pages e1007662.
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Romolo Nonno, Silvio Notari, Michele Angelo Di Bari, Ignazio Cali, Laura Pirisinu, Claudia d’Agostino, Laura Cracco, Diane Kofskey, Ilaria Vanni, Jody Lavrich, Piero Parchi, Umberto Agrimi & Pierluigi Gambetti. (2019) Variable Protease-Sensitive Prionopathy Transmission to Bank Voles. Emerging Infectious Diseases 25:1, pages 73-81.
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Aušrinė Areškevičiūtė, Linea Cecilie Melchior, Helle Broholm, Lars-Henrik Krarup, Suzanne Granhøj Lindquist, Peter Johansen, Neil McKenzie, Alison Green, Jørgen Erik Nielsen, Henning Laursen & Eva Løbner Lund. (2018) Sporadic Creutzfeldt-Jakob Disease in a Woman Married Into a Gerstmann-Sträussler-Scheinker Family: An Investigation of Prions Transmission via Microchimerism. Journal of Neuropathology & Experimental Neurology 77:8, pages 673-684.
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