The BAG2 and BAG5 proteins inhibit the ubiquitination of pathogenic ataxin3-80Q

被引：13

作者：

Che, Xiang-Qian ^{[1
]}

Tang, Bei-Sha ^{[1
,2
,3
,4
]}

Wang, Hong-Feng ^{[5
]}

Ya, Xin-Xiang ^{[1
,2
,3
,4
]}

Jiang, Hong ^{[1
,2
,3
,4
]}

Shen, Lu ^{[1
,2
,3
,4
]}

Xu, Qian ^{[1
,3
,4
]}

Wang, Guang-Hui ^{[5
]}

Zhang, Hai-Nan ^{[6
]}

Wang, Chun-Yu ^{[6
]}

Guo, Ji-Feng ^{[1
,2
,3
,4
]}

机构：

[1] Cent S Univ, Xiangya Hosp, Dept Neurol, Changsha 410008, Hunan, Peoples R China

[2] State Key Lab Med Genet, Changsha, Hunan, Peoples R China

[3] Cent S Univ, Neurodegenerat Disorders Res Ctr, Changsha 410008, Hunan, Peoples R China

[4] Cent S Univ, Human Key Lab Neurodegenerat Disorders, Changsha 410008, Hunan, Peoples R China

[5] Soochow Univ, Coll Pharmaceut Sci, Dept Pharmacol, Lab Mol Neuropathol, Suzhou, Jiangsu, Peoples R China

[6] Cent S Univ, Xiangya Hosp 2, Dept Neurol, Changsha 410008, Hunan, Peoples R China

来源：

INTERNATIONAL JOURNAL OF NEUROSCIENCE | 2015年 / 125卷 / 05期

基金：

中国国家自然科学基金;

关键词：

BAG2; BAG5; spinocerebellar ataxia type-3; pathogenic ataxin3-80Q; ubiquitin-proteasome system; MACHADO-JOSEPH-DISEASE; EXPANDED POLYGLUTAMINE PROTEIN; SPINOCEREBELLAR ATAXIA TYPE-3; HUNTINGTIN TOXICITY; PARKINSON-DISEASE; TRANSGENIC MICE; DEGRADATION; PATHWAY; TAU; AGGREGATION;

D O I：

10.3109/00207454.2014.940585

中图分类号：

Q189 [神经科学];

学科分类号：

071006 [神经生物学];

摘要：

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.

引用

页码：390 / 394

页数：5

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