Article
Cryo-electron tomography provides topological insights into mutant huntingtin exon 1 and polyQ aggregates.
Communications biology - 8 Jul 2021
Galaz-Montoya Jesús G, Shahmoradian Sarah H, Shen Koning, Frydman Judith, Chiu Wah
Abstract excerpt
Huntington disease (HD) is a neurodegenerative trinucleotide repeat disorder caused by an expanded poly-glutamine (polyQ) tract in the mutant huntingtin (mHTT) protein. The formation and topology of filamentous mHTT inclusions in the brain (hallmarks of HD implicated in neurotoxicity) remain elusive. Using cryo-electron tomography and subtomogram averaging, here we show that mHTT exon 1 and polyQ-only aggregates...
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