Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2024 Jul;47(7):538-550.
doi: 10.1016/j.tins.2024.05.004. Epub 2024 May 27.

Effect of host and strain factors on α-synuclein prion pathogenesis

Amanda L Woerman  1 , Jason C Bartz  2
Affiliations
Review

Effect of host and strain factors on α-synuclein prion pathogenesis

Amanda L Woerman et al. Trends Neurosci. 2024 Jul.

Abstract

Prion diseases are a group of neurodegenerative disorders caused by misfolding of proteins into pathogenic conformations that self-template to spread disease. Although this mechanism is largely associated with the prion protein (PrP) in classical prion diseases, a growing literature indicates that other proteins, including α-synuclein, rely on a similar disease mechanism. Notably, α-synuclein misfolds into distinct conformations, or strains, that cause discrete clinical disorders including multiple system atrophy (MSA) and Parkinson's disease (PD). Because the recognized similarities between PrP and α-synuclein are increasing, this review article draws from research on PrP to identify the host and strain factors that impact disease pathogenesis, predominantly in rodent models, and focuses on key considerations for future research on α-synuclein prions.

Keywords: Parkinson's disease; multiple system atrophy; neurodegenerative diseases; prion titer; strain biology; strain interactions.

PubMed Disclaimer

Conflict of interest statement

Declaration of interests A.L.W. is a coinventor on US patent application PCT/US2023/072173 which is related to some of the research discussed, and is a cofounder of Allagus Therapeutics. J.C.B. declares no competing interests in relation to this work.

Similar articles

See all similar articles

References

    1. Feigin VL et al. (2020) The global burden of neurological disorders: translating evidence into policy. Lancet Neurol 19 (3), 255–265. - PMC - PubMed
    1. Parkinson J., An essay on the shaking palsy, Sherwood, Neely, and Jones, London, 1817.
    1. Polymeropoulos MH et al. (1997) Mutation in the α-synuclein gene identified in families with Parkinson's disease. Science 276 (5321), 2045–2047. - PubMed
    1. Spillantini MG et al. (1997) α-Synuclein in Lewy bodies. Nature 388 (6645), 839–840. - PubMed
    1. Prusiner SB (1982) Novel proteinaceous infectious particles cause scrapie. Science 216, 136–144. - PubMed

LinkOut - more resources