Protein assembly disorders and protein-based inheritance

Aleksander A. Rubel, Alsu F. Saifitdinova, Nina V. Romanova

Research outputpeer-review

Abstract

Prions are self-perpetuating aggregated fibrous proteins that associated with fatal transmissible spongiform encephalopathies (TSEs) in mammals (including humans), and manifest themselves as non-Mendelian heritable elements in yeast and other fungi. The infectious agent responsible for TSEs is the prion, an abnormally folded and aggregated protein that propagates itself by imposing its conformation onto the cellular prion protein (PrPC) of the host. PrPC is necessary for prion replication and for prion-induced neurodegeneration, yet causes of neuronal injury and death are still poorly understood. Here we view of the prion concept, models describing of the replication and transport of prions particles, structural features and functions of the cellular PrP, the prion strain phenomenon, current developments in diagnostics of prion and potential antiprion therapies. Finally, we discuss how prion-like mechanisms may apply to other protein aggregation diseases.

Original languageEnglish
Title of host publicationGenetics, Evolution and Radiation
Subtitle of host publicationCrossing Borders, The Interdisciplinary Legacy of Nikolay W. Timofeeff-Ressovsky
PublisherSpringer
Pages85-105
Number of pages21
ISBN (Electronic)9783319488387
ISBN (Print)9783319488370
DOIs
Publication statusPublished - 1 Jan 2017

Fingerprint

Prions
prions
inheritance (genetics)
Proteins
protein
proteins
Prion Diseases
prion diseases
yeast
mammal
PrPC Proteins
fungus
Scleroproteins
Mammals
Fungi
Yeast
Conformations
Agglomeration
Yeasts
mammals

Scopus subject areas

  • Agricultural and Biological Sciences(all)
  • Engineering(all)
  • Environmental Science(all)
  • Medicine(all)
  • Biochemistry, Genetics and Molecular Biology(all)
  • Immunology and Microbiology(all)

Cite this

Rubel, A. A., Saifitdinova, A. F., & Romanova, N. V. (2017). Protein assembly disorders and protein-based inheritance. In Genetics, Evolution and Radiation: Crossing Borders, The Interdisciplinary Legacy of Nikolay W. Timofeeff-Ressovsky (pp. 85-105). Springer. https://doi.org/10.1007/978-3-319-48838-7_8
Rubel, Aleksander A. ; Saifitdinova, Alsu F. ; Romanova, Nina V. / Protein assembly disorders and protein-based inheritance. Genetics, Evolution and Radiation: Crossing Borders, The Interdisciplinary Legacy of Nikolay W. Timofeeff-Ressovsky. Springer, 2017. pp. 85-105
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Rubel, AA, Saifitdinova, AF & Romanova, NV 2017, Protein assembly disorders and protein-based inheritance. in Genetics, Evolution and Radiation: Crossing Borders, The Interdisciplinary Legacy of Nikolay W. Timofeeff-Ressovsky. Springer, pp. 85-105. https://doi.org/10.1007/978-3-319-48838-7_8

Protein assembly disorders and protein-based inheritance. / Rubel, Aleksander A.; Saifitdinova, Alsu F.; Romanova, Nina V.

Genetics, Evolution and Radiation: Crossing Borders, The Interdisciplinary Legacy of Nikolay W. Timofeeff-Ressovsky. Springer, 2017. p. 85-105.

Research outputpeer-review

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Rubel AA, Saifitdinova AF, Romanova NV. Protein assembly disorders and protein-based inheritance. In Genetics, Evolution and Radiation: Crossing Borders, The Interdisciplinary Legacy of Nikolay W. Timofeeff-Ressovsky. Springer. 2017. p. 85-105 https://doi.org/10.1007/978-3-319-48838-7_8