Genetic suppression of polyglutamine toxicity in Drosophila
Genetic suppression of polyglutamine toxicity in Drosophila
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DOI：
10.1126/science.287.5459.1837
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发表时间：
2000-03-10
期刊：
影响因子：
56.9
通讯作者：
Benzer, S
中科院分区：
文献类型：
--
作者：
Kazemi-Esfarjani, P;Benzer, S
亨廷顿氏症和其他聚谷氨酰胺病的果蝇模型被用来筛选改变眼睛中聚谷氨酰胺表达引起的退化的遗传因素。在7000个P元件插入中，分离到了几个抑制菌株，其中两个导致了这里描述的抑制基因的发现。其中一个的预测产物dHDJ1与人类热休克蛋白40/HDJ1同源。第二个是dTPR2，与人的四肽重复蛋白2同源。每个分子都含有一个伴侣相关的J结构域。它们对多聚谷氨酰胺毒性的抑制在转基因果蝇中得到了验证。
A Drosophila model for Huntington's and other polyglutamine diseases was used to screen for genetic factors modifying the degeneration caused by expression of polyglutamine in the eye. Among 7000 P-element insertions, several suppressor strains were isolated, two of which Led to the discovery of the suppressor genes described here. The predicted product of one, dHDJ1, is homologous to human heat shock protein 40/HDJ1. That of the second, dTPR2, is homologous to the human tetratricopeptide repeat protein 2. Each of these molecules contains a chaperone-related J domain. Their suppression of polyglutamine toxicity was verified in transgenic flies.