Kaleidoscopic protein-protein interactions in the life and death of ataxin-1: new strategies against protein aggregation.
Kaleidoscopic protein-protein interactions in the life and death of ataxin-1: new strategies against protein aggregation.
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DOI：
10.1016/j.tins.2014.02.003
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发表时间：
2014-04
影响因子：
15.9
通讯作者：
Pastore A
中科院分区：
文献类型：
--
作者：
de Chiara C;Pastore A
Ataxin-1（Atx 1）是脊髓小脑性共济失调1型（SCA 1）的致病蛋白。正常功能和异常聚集是竞争途径。蛋白质-蛋白质相互作用保护Atx 1免于聚集和错误折叠。这些知识可以用于药物开发。了解蛋白质如何保护自己免受异常聚集的影响，对于理解基础生物学、蛋白质生物化学和人类疾病具有重要意义。我们讨论了典型的例子，共济失调蛋白-1（Atx 1），负责神经退行性脊髓小脑共济失调1型（SCA 1）的蛋白质。这种疾病是由蛋白质聚集和错误折叠引起的日益增加的病理家族的一部分。我们讨论的重要性，蛋白质-蛋白质相互作用不仅在非病理功能的Atx 1，但也在保护蛋白质聚集和错误折叠。从Atx 1中吸取的教训可能会导致对细胞抗聚集保护策略的更普遍的理解。所获得的知识可能为设计治疗错误折叠疾病的特定治疗策略提供新的视角。
Ataxin-1 (Atx1) is the protein responsible for spinocerebellar ataxia type 1 (SCA1). Normal function and anomalous aggregation are competing pathways. Protein–protein interactions protect Atx1 from aggregation and misfolding. This knowledge can be exploited in drug development. Understanding how proteins protect themselves from aberrant aggregation is of primary interest for understanding basic biology, protein biochemistry, and human disease. We discuss the paradigmatic example of ataxin-1 (Atx1), the protein responsible for neurodegenerative spinocerebellar ataxia type 1 (SCA1). This disease is part of the increasing family of pathologies caused by protein aggregation and misfolding. We discuss the importance of protein–protein interactions not only in the nonpathological function of Atx1 but also in protecting the protein from aggregation and misfolding. The lessons learned from Atx1 may lead to a more general understanding of the cell's protective strategies against aggregation. The obtained knowledge may suggest a new perspective for designing specific therapeutic strategies for the cure of misfolding diseases.
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