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Updated: Jul 13, 2026

Cerebellar Regional Dissection for Molecular Analysis
Published on: December 5, 2020
Inclusion formation by ataxins -1, -2, -3, and -7
Volga Tarlac1, Victor Turnbull, Daniela Stefani
1Van Cleef Roet Centre for Nervous Diseases, Department of Medicine (Neuroscience), Monash University (Alfred Hospital Campus), Melbourne, VIC, Australia.
Abstract:
The authors studied inclusion formation in vitro using transiently transfected PC12 cells, with epitope-tagged and untagged full-length and truncated wild-type and expanded ataxins -1, -2, -3, and -7. At 72 hours, no inclusions were seen with wild-type full-length or truncated ataxins -2, -3, or -7, and only one with ataxin-1. Truncation abolished nuclear localization of ataxins -1 and -7, and allowed nuclear entry of ataxin-2. Of the expanded ataxins, only -1 and -2 formed inclusions, and those of ataxin-2 were rare and exclusively cytoplasmic. Truncation resulted in inclusion formation by ataxins -3 and -7, increased ataxin-1 inclusions, and enabled formation of nuclear ataxin-2 inclusions. There was no recruitment of wild-type ataxin-1 to expanded ataxin-1 inclusions.
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