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
. 2021 Sep;268(9):3493-3504.
doi: 10.1007/s00415-021-10418-8. Epub 2021 Feb 24.

Huntington's disease: lessons from prion disorders

Melanie Alpaugh  1   2 , Francesca Cicchetti  3   4
Affiliations

Huntington's disease: lessons from prion disorders

Melanie Alpaugh et al. J Neurol. 2021 Sep.

Abstract

Decades of research on the prion protein and its associated diseases have caused a paradigm shift in our understanding of infectious agents. More recent years have been marked by a surge of studies supporting the application of these findings to a broad array of neurodegenerative disorders such as Alzheimer's and Parkinson's diseases. Here, we present evidence to suggest that Huntington's disease, a monogenic disorder of the central nervous system, shares features with prion disorders and that, it too, may be governed by similar mechanisms. We further posit that these similarities could suggest that, like other common neurodegenerative disorders, sporadic forms of Huntington's disease may exist.

Keywords: Huntingtin; Huntington’s disease-like; Prion-like; Tissue spongiform encephalopathies.

PubMed Disclaimer

References

    1. Babcock DT, Ganetzky B (2015) Transcellular spreading of huntingtin aggregates in the Drosophila brain. PNAS 112(39):E5427–E5433. https://doi.org/10.1073/pnas.1516217112 - DOI - PubMed - PMC
    1. Masnata M, Sciacca G, Maxan A, Bousset L, Denis HL, Lauruol F, David L, Saint-Pierre M, Kordower JH, Melki R, Alpaugh M, Cicchetti F (2019) Demonstration of prion-like properties of mutant huntingtin fibrils in both in vitro and in vivo paradigms. Acta Neuropathol 137(6):981–1001. https://doi.org/10.1007/s00401-019-01973-6 - DOI - PubMed - PMC
    1. Gosset P, Maxan A, Alpaugh M, Breger L, Dehay B, Tao Z, Ling Z, Qin C, Cisbani G, Fortin N, Vonsattel JG, Lacroix S, Oueslati A, Bezard E, Cicchetti F (2020) Evidence for the spread of human-derived mutant huntingtin protein in mice and non-human primates. Neurobiol Dis 141:104941. https://doi.org/10.1016/j.nbd.2020.104941 - DOI - PubMed
    1. Rieux M, Alpaugh M, Sciacca G, Saint-Pierre M, Masnata M, Denis HL, Levesque SA, Herrmann F, Bazenet C, Garneau AP, Isenring P, Truant R, Oueslati A, Gould PV, Ast A, Wanker EE, Lacroix S, Cicchetti F (2020) Shedding a new light on Huntington's disease: how blood can both propagate and ameliorate disease pathology. Mol Psychiatry. Epub ahead of print. https://doi.org/10.1038/s41380-020-0787-4 . Erratum in: Mol Psychiatry. 2020 Jul 24: PMID: 32514103
    1. Jeon I, Cicchetti F, Cisbani G, Lee S, Li E, Bae J, Lee N, Li L, Im W, Kim M, Kim HS, Oh SH, Kim TA, Ko JJ, Aube B, Oueslati A, Kim YJ, Song J (2016) Human-to-mouse prion-like propagation of mutant huntingtin protein. Acta Neuropathol 132(4):577–592. https://doi.org/10.1007/s00401-016-1582-9 - DOI - PubMed - PMC

LinkOut - more resources