| 引用本文: | 李羽彬,严芳洁,何俏军,杨波,朱虹.去泛素化酶Machado-joseph家族蛋白在疾病中的功能研究进展[J].中国现代应用药学,2019,36(6):757-762. |
| LI Yubin,YAN Fangjie,HE Qiaojun,YANG Bo,ZHU Hong.Research Progress on the Function of Machado-joseph Family Members of Deubiquitinating Enzymes in Diseases[J].Chin J Mod Appl Pharm(中国现代应用药学),2019,36(6):757-762. |
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| 摘要: |
| 去泛素化酶在维持蛋白的稳定性和功能中发挥重要作用，因此是调控多种重要生命活动过程的关键因素，Machado-josep（MJD）家族作为6个去泛素化酶家族之一，其蛋白包括Ataxin-3、ATAN3L、JOSD1和JOSD2，在人体各个器官部位较为广泛地表达，其中，Ataxin-3与神经退行性疾病MJD疾病也密切相关。近年来，针对MJD家族蛋白在生物学、相关疾病方面的研究日益增多，本文将对近年来MJD家族蛋白的相关研究进展进行综述。 |
| 关键词: 去泛素化酶 Machado-joseph家族 Ataxin-3 疾病 |
| DOI：10.13748/j.cnki.issn1007-7693.2019.06.024 |
| 分类号:R963 |
| 基金项目: |
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| Research Progress on the Function of Machado-joseph Family Members of Deubiquitinating Enzymes in Diseases |
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LI Yubin, YAN Fangjie, HE Qiaojun, YANG Bo, ZHU Hong
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College of Pharmaceutical Sciences, Zhejiang University, Hangzhou 310058, China
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| Abstract: |
| The deubiquitinating enzymes(DUBs) play important roles in a variety of physiological process. The Machado-joseph(MJD) family is one of the six DUBs families. The proteins of maintaining of stability and function of proteins which are clinical factors including Ataxin-3, ATAN3L, JOSD1 and JOSD2, which are widely expressed in various parts of the human body. Among them, Ataxin-3 is also closely related to the MJD disease, a neurodegenerative disease. Recently, the biological function as well as the roles in the progression of diseases of the MJD family members have been extensively studied. This review will summarize these research progress of MJD family members in recent years. |
| Key words: deubiquitinating enzymes Machado-joseph(MJD) family Ataxin-3 disease |
