Department of Anatomy, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Neurosci Lett. 2012 May 16;516(2):182-7. doi: 10.1016/j.neulet.2012.03.074. Epub 2012 Apr 2.
The ER-localized chaperone glucose-regulated protein (GRP78) protects neurons against excitotoxicity and apoptosis. Here we show that overexpressing GRP78 protects N2a cells against mutant huntingtin proteins, reduces formation of mutant huntingtin aggregates, inhibits caspase-12 activation and blocks cell death. Our data suggest that GRP78 may be a promising therapeutic target for the treatment of Huntington's disease.
内质网定位伴侣蛋白葡萄糖调节蛋白（GRP78）可保护神经元免受兴奋毒性和细胞凋亡的影响。本文中我们发现，过表达 GRP78 可保护 N2a 细胞免受突变型 huntingtin 蛋白的影响，减少突变型 huntingtin 聚集物的形成，抑制半胱天冬酶-12 的激活并阻止细胞死亡。我们的数据表明，GRP78 可能是治疗亨廷顿病的有希望的治疗靶点。
