Tarlac Volga, Turnbull Victor, Stefani Daniela, Kelly Louise, Walsh Renae, Storey Elsdon
Van Cleef Roet Centre for Nervous Diseases, Department of Medicine (Neuroscience), Monash University (Alfred Hospital Campus), Melbourne, VIC, Australia.
Int J Neurosci. 2007 Sep;117(9):1289-314. doi: 10.1080/00207450600936668.
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.
作者使用瞬时转染的PC12细胞在体外研究包涵体形成，所用的蛋白包括带有表位标签和未带标签的全长及截短的野生型和扩展型ataxin -1、-2、-3和-7。72小时时，野生型全长或截短的ataxin -2、-3或-7未观察到包涵体，ataxin-1仅观察到一个。截短消除了ataxin -1和-7的核定位，并使ataxin-2能够进入细胞核。在扩展型ataxins中，只有-1和-2形成包涵体，且ataxin-2的包涵体很少且仅存在于细胞质中。截短导致ataxin -3和-7形成包涵体，增加了ataxin-1包涵体的形成，并使细胞核中形成ataxin-2包涵体。野生型ataxin-1不会被招募到扩展型ataxin-1包涵体中。
