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-细胞与分子生物学研究-CSCIED科技核心评价数据库-手机版

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

JJS activates the ubiquitin protease system to degrade Huntingtons disease-associated misfolded proteins

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DOI	10.12208/j.jcmbr.20230003
刊名	细胞与分子生物学研究 Journal of Cell and Molecular Biology Research
年，卷(期)	2023, 3(1)
作者	潘蓉,冼桂羽,卿即娜*,
作者单位	长沙医学院口腔医学院湖南长沙 ;
摘要	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。
Abstract	Objective To investigate in depth the role of JJS in inhibiting Huntingtons 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.
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
KeyWord	Huntingtons disease; JJS; Huntingtons disease-associated pathogenic protein; ubiquitin-proteasome pathway
基金项目
页码	9-13

参考文献
相关文献

[1] 闫森.亨廷顿舞蹈病神经发育障碍的研究进展
[J].中山大学学报(医学科学版),2022,43(05):691-696.

[2] 林丽山，苏凤娟，吴腾腾，等. 中国南方地区亨廷顿患者不同运动分型的临床特征［J］. 中山大学学报（医学科学版），2021，42（6）：944-949.

[3] 张元,郭晓红,刘国荣，等.亨廷顿病一家系报道及相关文献回顾分析
[J].中风与神经疾病杂志,2022,39(01):78-80.

[4] 唐一鸣,姚逸飞,杨中元等.神经退行性疾病相关蛋白病理性聚集和液液相分离研究进展
[J].合成生物学,2023,4(03):590-610.

[5] 熊朝栋. 基于泛素—蛋白酶体系统（UPS）的新型抑制剂的设计、合成及抗肿瘤活性研究
[D].中国科学院大学(中国科学院上海药物研究所),2022.

[6] 郭园园,郑琴,胡鹏翼等.中药调控能量代谢治疗神经退行性疾病的研究进展
[J].中药新药与临床药理, 2020, 31(11):1384-1388.

[7] 应春苗,刘飞祥,潘小龙等.中药延缓神经血管单元衰老治疗神经退行性疾病的研究进展
[J/OL].中国中药杂志:1-12
[2023-07-26].

[8] Law B Y K, Wu A G, Wang M J, et al. Chinese Medicine: A Hope for Neurodegenerative Diseases?
[J]. J Alzheimers Dis, 2017, 60(s1): S151-S60.

[9] Sheikh S, Safia, Haque E, et al. Neurodegenerative Diseases: Multifactorial Conformational Diseases and Their Therapeutic Interventions
[J]. J Neurodegener Dis, 2013, 2013(563481.

[10] Popovic D, Vucic D, Dikic I. Ubiquitination in disease pathogenesis and treatment
[J]. Nat Med, 2014, 20(11): 1242-53.

[11] Fu Y, Sun X, Lu B. HIPK3 modulates autophagy and HTT protein levels in neuronal and mouse models of Huntington disease
[J]. Autophagy, 2018, 14(1): 169-70.

[12] Veldman M B, Yang X W. Molecular insights into cortico-striatal miscommunications in Huntington's disease
[J]. Curr Opin Neurobiol, 2018, 48(79-89.

[13] Yu M, Fu Y, Liang Y, et al. Suppression of MAPK11 or HIPK3 reduces mutant Huntingtin levels in Huntington's disease models
[J]. Cell Res, 2017, 27(12): 1441-65.

[14] Zhao T, Hong Y, Li X J, et al. Subcellular Clearance and Accumulation of Huntington Disease Protein: A Mini-Review
[J]. Front Mol Neurosci, 2016, 9(27.

[15] Zhou X, Li G, Kaplan A, et al. Small molecule modulator of protein disulfide isomerase attenuates mutant huntingtin toxicity and inhibits endoplasmic reticulum stress in a mouse model of Huntington's disease
[J]. Hum Mol Genet, 2018, 27(9): 1545-55.

[16] Liu Q, Chen Y, Shen C, et al. Chicoric acid supplementation prevents systemic inflammation-induced memory impairment and amyloidogenesis via inhibition of NF-kappaB
[J]. FASEB J, 2017, 31(4): 1494-507.

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.

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