Roles of Post-translational Modifications in Spinocerebellar Ataxias.

Roles of Post-translational Modifications in Spinocerebellar Ataxias.
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翻译后修饰在脊髓小脑共济失调中的作用。

DOI:
10.3389/fncel.2018.00290
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发表时间:
2018
影响因子:
5.3
通讯作者:
Jiang H
Jiang H
中科院分区:
医学2区
文献类型:
--
作者:
Wan L;Xu K;Chen Z;Tang B;Jiang H

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蛋白质的翻译后修饰(PTM)包括磷酸化、乙酰化、泛素化、SUMO化等,可以调节蛋白质在细胞内的分布、活性、稳定性、聚集和相互作用等性质。因此,PTMS是多种细胞过程的重要调节机制。脊髓小脑性共济失调(SCA)是一种遗传性、异质性、神经退行性疾病,其主要表现为共济失调。由于大多数SCA的发病机制与突变蛋白直接或间接相关,疾病相关蛋白的PTM可能在功能上影响SCA的发展,是潜在的治疗干预措施。在此,我们对多种与致病蛋白相关的PTM在SCAS发病机制中的作用进行综述。此外,我们讨论了这些PTM作为治疗SCA的潜在靶点,并描述了已发表的针对PTM的翻译疗法。
Post-translational modifications (PTMs), including phosphorylation, acetylation, ubiquitination, SUMOylation, etc., of proteins can modulate protein properties such as intracellular distribution, activity, stability, aggregation, and interactions. Therefore, PTMs are vital regulatory mechanisms for multiple cellular processes. Spinocerebellar ataxias (SCAs) are hereditary, heterogeneous, neurodegenerative diseases for which the primary manifestation involves ataxia. Because the pathogenesis of most SCAs is correlated with mutant proteins directly or indirectly, the PTMs of disease-related proteins might functionally affect SCA development and represent potential therapeutic interventions. Here, we review multiple PTMs related to disease-causing proteins in SCAs pathogenesis and their effects. Furthermore, we discuss these PTMs as potential targets for treating SCAs and describe translational therapies targeting PTMs that have been published.
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