Ubiquitination is a post-translational modification that can dynamically alter the function, degradation and transport of a protein, as well as its interaction with other proteins, and activity of an enzyme. Dysfunctional ubiquitination is detrimental to normal cellular functions, and can result in severe diseases. Over the last decade, although much research has focused on deciphering the role of the ubiquitination/ubiquitin proteasome system (UPS) in the onset and progression of various neurological disorders, the specific relationship between ubiquitination and various epilepsies has not been carefully reviewed. As an increasing amount of research has revealed the roles of ubiquitination in the trafficking of ion channels and the turn-over of synaptic receptors, it is crucial to take a deeper look into ubiquitination-associated epilepsy. Here, we review the role of ubiquitination in maintaining normal cellular activities in neurons and recent findings on the dysregulation of ubiquitination in epilepsy. We particularly focus on rare neurological disorders with comorbid epilepsy in the hope of drawing more attention to this area. Through categorizing epilepsy-associated E3 ubiquitin ligases and their substrates and discussing ubiquitination-related rare neurological disorders, we summarize where the field stands at the moment and what directions we should consider in the future.

中文翻译：

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罕见的神经系统疾病合并并发癫痫中的泛素化和E3泛素天冬氨酸。

泛素化是翻译后修饰，可以动态改变蛋白质的功能，降解和运输，以及其与其他蛋白质的相互作用以及酶的活性。功能失调的泛素化对正常的细胞功能有害，并可能导致严重的疾病。在过去的十年中，尽管许多研究集中在破译泛素化/泛素蛋白酶体系统（UPS）在各种神经系统疾病的发作和发展中的作用，但泛素化与各种癫痫之间的具体关系尚未得到仔细审查。随着越来越多的研究表明泛素化在离子通道运输和突触受体转换中的作用，对泛素化相关性癫痫病的深入研究至关重要。这里，我们综述了泛素化在维持神经元正常细胞活动中的作用以及有关癫痫中泛素化失调的最新发现。我们特别关注合并症癫痫的罕见神经系统疾病，希望引起更多关注。通过对与癫痫相关的E3泛素连接酶及其底物进行分类，并讨论与泛素化相关的罕见神经系统疾病，我们总结了目前的领域以及未来应考虑的方向。