Soares Ericks S, Prediger Rui D, Brocardo Patricia S, Cimarosti Helena I
Post-graduate Program in Pharmacology, Federal University of Santa Catarina (UFSC), Florianópolis, Santa Catarina, Brazil.
Post-graduate Program in Neuroscience, UFSC, Florianópolis, Santa Catarina, Brazil.
IBRO Neurosci Rep. 2022 Mar 9;12:203-209. doi: 10.1016/j.ibneur.2022.03.002. eCollection 2022 Jun.
Small ubiquitin-like modifiers, SUMOs, are proteins that are conjugated to target substrates and regulate their functions in a post-translational modification called SUMOylation. In addition to its physiological roles, SUMOylation has been implicated in several neurodegenerative diseases, such as Alzheimer's, Parkinson's, and Huntington's diseases (HD). HD is a neurodegenerative monogenetic autosomal dominant disorder caused by a mutation in the CAG repeat of the huntingtin () gene, which expresses a mutant Htt protein more susceptible to aggregation and toxicity. Besides Htt, other SUMO ligases, enzymes, mitochondrial and autophagic components are also important for the progression of the disease. Here we review the main aspects of Htt SUMOylation and its role in cellular processes involved in the pathogenesis of HD.
小泛素样修饰物（SUMO）是一类蛋白质，它们与靶底物结合，并在一种称为SUMO化的翻译后修饰过程中调节底物的功能。除了其生理作用外，SUMO化还与多种神经退行性疾病有关，如阿尔茨海默病、帕金森病和亨廷顿舞蹈病（HD）。HD是一种神经退行性单基因常染色体显性疾病，由亨廷顿蛋白（Htt）基因CAG重复序列突变引起，该基因表达的突变型Htt蛋白更易聚集并具有毒性。除Htt外，其他SUMO连接酶、酶、线粒体和自噬成分对该疾病的进展也很重要。在此，我们综述了Htt SUMO化的主要方面及其在HD发病机制相关细胞过程中的作用。
