Alpha-synuclein immunoreactivity of huntingtin polyglutamine aggregates in striatum and cortex of Huntington's disease patients and transgenic mouse models

Vinod Charles, Eva Mezey, P. Hemachandra Reddy, Anindya Dehejia, Theresa A. Young, Mihail H. Polymeropoulos, Michael J. Brownstein, Danilo A. Tagle

    Research output: Contribution to journalArticle

    48 Scopus citations

    Abstract

    Polyglutamine expansions in proteins are implicated in at least eight inherited neurodegenerative disorders, including Huntington's disease. These mutant proteins can form aggregates within the nucleus and processes of neurons possibly due to misfolding of the proteins. Polyglutamine aggregates are ubiquitinated and sequester molecular chaperone proteins and proteasome components. To investigate other protein components of polyglutamine aggregates, cerebral cortex and striata from patients with Huntington's disease and full-length cDNA transgenic mouse models for this disease were examined immunohistochemically for α-synuclein reactivity. Our findings demonstrate that α-synuclein can be used as a marker for huntingtin polyglutamine aggregates in both human and mice. Moreover in the HD transgenic mice, the intensity of immunoreactivity increases with age. The significance of recruitment of α-synuclein into huntingtin aggregates and its translocation away from the synapses remains to be determined. We propose that aberrant interaction of mutant huntingtin with other proteins, including α-synuclein, may influence disease progression. Copyright (C) 2000 Elsevier Science Ireland Ltd.

    Original languageEnglish (US)
    Pages (from-to)29-32
    Number of pages4
    JournalNeuroscience Letters
    Volume289
    Issue number1
    DOIs
    StatePublished - Jul 28 2000

    Keywords

    • Cerebral cortex
    • Huntingtin
    • Huntington's disease
    • Immunohistochemistry
    • Parkinson's disease
    • Striatum
    • Transgenic mice
    • α-Synuclein

    ASJC Scopus subject areas

    • Neuroscience(all)

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  • Cite this

    Charles, V., Mezey, E., Reddy, P. H., Dehejia, A., Young, T. A., Polymeropoulos, M. H., Brownstein, M. J., & Tagle, D. A. (2000). Alpha-synuclein immunoreactivity of huntingtin polyglutamine aggregates in striatum and cortex of Huntington's disease patients and transgenic mouse models. Neuroscience Letters, 289(1), 29-32. https://doi.org/10.1016/S0304-3940(00)01247-7