The BAG2 and BAG5 proteins inhibit the ubiquitination of pathogenic ataxin3-80Q
The BAG2 and BAG5 proteins inhibit the ubiquitination of pathogenic ataxin3-80Q
复制标题
BAG2 和 BAG5 蛋白抑制致病性 ataxin3-80Q 的泛素化
DOI：
10.3109/00207454.2014.940585
复制
发表时间：
2015-05-01
影响因子：
2.2
通讯作者：
Guo，Ji-Feng
中科院分区：
文献类型：
--
作者：
Che，Xiang-Qian;Tang，Bei-Sha;Guo，Ji-Feng
蛋白ataxin3中聚谷氨酰胺结构域的扩张导致脊髓小脑性共济失调3型（SCA3）。然而，迄今为止关于致病性ataxin3-80Q泛素-蛋白酶体系统上游蛋白的信息很少。在这里，我们报道了BAG2 （Bcl-2相关thanogene家族蛋白2）和BAG5 （Bcl-2相关thanogene家族蛋白5）通过抑制其泛素化来稳定致病性ataxin3-80Q，这是基于western blotting和共免疫荧光实验确定的。BAG2和BAG5蛋白与致病性ataxin3-80Q的关联加强了BAG家族在神经退行性疾病中的重要作用。
The expansion of a polyglutamine domain in the protein ataxin3 causes spinocerebellar ataxia type-3 (SCA3). However, there is little information to date about the upstream proteins in the ubiquitin-proteasome system of pathogenic ataxin3-80Q. Here, we report that BAG2 (Bcl-2 associated athanogene family protein 2) and BAG5 (Bcl-2-associated athanogene family protein 5) stabilise pathogenic ataxin3-80Q by inhibiting its ubiquitination as determined based on western blotting and co-immunofluorescence experiments. The association of the BAG2 and BAG5 proteins with pathogenic ataxin3-80Q strengthens the important roles of the BAG family in neurodegenerative diseases.