Article
Disease-related Huntingtin seeding activities in cerebrospinal fluids of Huntington's disease patients.
Scientific reports - 20 Nov 2020
Lee C Y Daniel, Wang Nan, Shen Koning, Stricos Matthew, Langfelder Peter, Cheon Kristina H, Cortés Etty P, Vinters Harry V, Vonsattel Jean Paul, Wexler Nancy S, Damoiseaux Robert, Frydman Judith, Yang X William
Abstract excerpt
In Huntington's disease (HD), the mutant Huntingtin (mHTT) is postulated to mediate template-based aggregation that can propagate across cells. It has been difficult to quantitatively detect such pathological seeding activities in patient biosamples, e.g. cerebrospinal fluids (CSF), and study their correlation with the disease manifestation. Here we developed a cell line expressing a domain-engineered mHTT-exon 1...
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