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Open Access Article

Journal of Cell and Molecular Biology Research. 2023; 3: (1) ; 9-13 ; DOI: 10.12208/j.jcmbr.20230003.

JJS activates the ubiquitin protease system to degrade Huntington's disease-associated misfolded proteins
JJS激活泛素蛋白酶系统降解亨廷顿病相关的错误折叠蛋白

作者: 潘蓉, 冼桂羽, 卿即娜 *

长沙医学院口腔医学院 湖南长沙

*通讯作者: 卿即娜,单位:长沙医学院口腔医学院 湖南长沙;

发布时间: 2023-09-20 总浏览量: 818

摘要

目的 深入探讨JJS抑制亨廷顿病(HD)相关的致病蛋白(mHTT)和激活泛素-蛋白酶体途径(UPS)系统的作用,进一步开发JJS的功效。方法 通过构建神经退行性疾病相关的HD和UPS调控的细胞模型,使用细胞免疫荧光、流式细胞法和免疫印迹法检测不同浓度JJS处理的细胞中mHTT的表达。结果 JJS激活UPS减少HT22细胞中EGFP-mHTT74的过表达。结论 JJS可以通过激活UPS抑制mHTT。

关键词: 亨廷顿病;JJS;亨廷顿病相关致病蛋白;泛素-蛋白酶体途径

Abstract

Objective To investigate in depth the role of JJS in inhibiting Huntington's disease (HD)-associated pathogenic proteins (mHTT) and activating the ubiquitin-proteasome pathway (UPS) system, and to further develop the efficacy of JJS.
Methods By constructing a cellular model of neurodegenerative disease-associated HD and UPS regulation, the expression of mHTT in cells treated with different concentrations of JJS was examined using cellular immunofluorescence, flow cytometry and immunoblotting.
Results JJS activation of UPS reduced overexpression of EGFP-mHTT74 in HT22 cells.
Conclusion   JJS can inhibit mHTT by activating UPS.

Key words: Huntington's disease; JJS; Huntington's disease-associated pathogenic protein; ubiquitin-proteasome pathway

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引用本文

潘蓉, 冼桂羽, 卿即娜, JJS激活泛素蛋白酶系统降解亨廷顿病相关的错误折叠蛋白[J]. 细胞与分子生物学研究, 2023; 3: (1) : 9-13.