4.7 Article

Huntington's disease: lessons from prion disorders

期刊

JOURNAL OF NEUROLOGY
卷 268, 期 9, 页码 3493-3504

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SPRINGER HEIDELBERG
DOI: 10.1007/s00415-021-10418-8

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

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Research suggests that Huntington's disease shares similarities with prion disorders and may also have sporadic forms, indicating potential common mechanisms governing these neurodegenerative disorders.
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.

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