The prion model for progression and diversity of neurodegenerative diseases - PubMed (original) (raw)

Review

. 2017 Apr;16(4):323-332.

doi: 10.1016/S1474-4422(17)30037-6. Epub 2017 Feb 24.

Affiliations

• PMID: 28238712
• DOI: 10.1016/S1474-4422(17)30037-6

Review

The prion model for progression and diversity of neurodegenerative diseases

Barbara E Stopschinski et al. Lancet Neurol. 2017 Apr.

Abstract

The neuropathology of different neurodegenerative diseases begins in different brain regions, and involves distinct brain networks. Evidence indicates that transcellular propagation of protein aggregation, which is the basis of prion disease, might underlie the progression of pathology in neurodegenerative diseases such as Alzheimer's disease, Parkinson's disease, and Huntington's disease. The prion model predicts specific patterns of neuronal vulnerability and network involvement on the basis of the conformation of pathological proteins. Indeed, evidence indicates that self-propagating aggregate conformers, or so-called strains, are associated with distinct neuropathological syndromes. The extension of this hypothesis to our understanding of common neurodegenerative disorders can suggest new therapeutic approaches, such as immunotherapy and small molecules, to block transcellular propagation, and new diagnostic tools to detect early evidence of disease.

Copyright © 2017 Elsevier Ltd. All rights reserved.

PubMed Disclaimer

Comment in

• Solving the conundrum of insoluble protein aggregates.
  George S, Brundin P. George S, et al. Lancet Neurol. 2017 Apr;16(4):258-259. doi: 10.1016/S1474-4422(17)30045-5. Epub 2017 Feb 24. Lancet Neurol. 2017. PMID: 28238710 No abstract available.

Similar articles

• Propagation of Protein Aggregation in Neurodegenerative Diseases.
  Vaquer-Alicea J, Diamond MI. Vaquer-Alicea J, et al. Annu Rev Biochem. 2019 Jun 20;88:785-810. doi: 10.1146/annurev-biochem-061516-045049. Epub 2019 Mar 27. Annu Rev Biochem. 2019. PMID: 30917002 Review.
• [The Propagation Hypothesis of Prion-like Protein Agregates in Neurodegenerative Diseases].
  Nonaka T. Nonaka T. Brain Nerve. 2019 Nov;71(11):1209-1214. doi: 10.11477/mf.1416201430. Brain Nerve. 2019. PMID: 31722306 Japanese.
• POSCAbilities: The Application of the Prion Organotypic Slice Culture Assay to Neurodegenerative Disease Research.
  Pineau H, Sim V. Pineau H, et al. Biomolecules. 2020 Jul 20;10(7):1079. doi: 10.3390/biom10071079. Biomolecules. 2020. PMID: 32698402 Free PMC article. Review.
• Generalization of the prion hypothesis to other neurodegenerative diseases: an imperfect fit.
  Guest WC, Silverman JM, Pokrishevsky E, O'Neill MA, Grad LI, Cashman NR. Guest WC, et al. J Toxicol Environ Health A. 2011;74(22-24):1433-59. doi: 10.1080/15287394.2011.618967. J Toxicol Environ Health A. 2011. PMID: 22043906 Review.
• Invited Review: The role of prion-like mechanisms in neurodegenerative diseases.
  Jaunmuktane Z, Brandner S. Jaunmuktane Z, et al. Neuropathol Appl Neurobiol. 2020 Oct;46(6):522-545. doi: 10.1111/nan.12592. Epub 2020 Feb 11. Neuropathol Appl Neurobiol. 2020. PMID: 31868945 Free PMC article. Review.

Cited by

• A Drosophila model for mechanistic investigation of tau protein spread.
  Bankapalli K, Thomas RE, Vincow ES, Milstein G, Fisher LV, Pallanck LJ. Bankapalli K, et al. Dis Model Mech. 2024 Sep 1;17(9):dmm050858. doi: 10.1242/dmm.050858. Epub 2024 Oct 1. Dis Model Mech. 2024. PMID: 39350752 Free PMC article.
• Lipid signaling to membrane proteins: From second messengers to membrane domains and adapter-free endocytosis.
  Hilgemann DW, Dai G, Collins A, Lariccia V, Magi S, Deisl C, Fine M. Hilgemann DW, et al. J Gen Physiol. 2018 Feb 5;150(2):211-224. doi: 10.1085/jgp.201711875. Epub 2018 Jan 11. J Gen Physiol. 2018. PMID: 29326133 Free PMC article. Review.
• Longitudinal accrual of neocortical amyloid burden is associated with microstructural changes of the fornix in cognitively normal adults.
  Song Z, Farrell ME, Chen X, Park DC. Song Z, et al. Neurobiol Aging. 2018 Aug;68:114-122. doi: 10.1016/j.neurobiolaging.2018.02.021. Epub 2018 Mar 6. Neurobiol Aging. 2018. PMID: 29602495 Free PMC article.
• Exosomal tau with seeding activity is released from Alzheimer's disease synapses, and seeding potential is associated with amyloid beta.
  Miyoshi E, Bilousova T, Melnik M, Fakhrutdinov D, Poon WW, Vinters HV, Miller CA, Corrada M, Kawas C, Bohannan R, Caraway C, Elias C, Maina KN, Campagna JJ, John V, Gylys KH. Miyoshi E, et al. Lab Invest. 2021 Dec;101(12):1605-1617. doi: 10.1038/s41374-021-00644-z. Epub 2021 Aug 30. Lab Invest. 2021. PMID: 34462532 Free PMC article.
• Modeling neurodegenerative diseases with cerebral organoids and other three-dimensional culture systems: focus on Alzheimer's disease.
  Venkataraman L, Fair SR, McElroy CA, Hester ME, Fu H. Venkataraman L, et al. Stem Cell Rev Rep. 2022 Feb;18(2):696-717. doi: 10.1007/s12015-020-10068-9. Epub 2020 Nov 12. Stem Cell Rev Rep. 2022. PMID: 33180261 Free PMC article. Review.

Publication types

MeSH terms

LinkOut - more resources

• Full Text Sources

  • ClinicalKey
  • Elsevier Science

• Other Literature Sources

  • The Lens - Patent Citations Database
  • scite Smart Citations

• Medical

  • MedlinePlus Health Information