Abstract

Huntington disease (HD) is a hereditary neurodegenerative disease that results in impairment of brain function. HD is associated with expanded trinucleotide repeats in the huntingtin (HTT) gene, which translate into polyglutamine (polyQ) stretches within the huntingtin protein (Htt). Researchers have hypothesized that expanded polyQ domains promote aggregation of Htt, which leads to neurodegeneration in HD patients. In the January 18 issue of the Proceedings of the National Academy of Sciences, Zhang et al. identify organic compounds that inhibit polyQ aggregation in brain slices and block their neurodegenerative effects in a Drosophila melanogaster model of HD.

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