Inclusion formation by ataxins -1, -2, -3, and -7

Volga Tarlac, Victor John Turnbull, Daniela Stefani, Louise Kelly, Renae Walsh, Elsdon Storey

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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.
Original languageEnglish
Pages (from-to)1289 - 1314
Number of pages26
JournalInternational Journal of Neuroscience
Issue number9
Publication statusPublished - 2007

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