Department of Pharmacology, Faculty of Medicine, Chiang Mai University, Chiang Mai 50200, Thailand.
Department of Neurotherapeutics, Osaka University Graduate School of Medicine, Osaka 565-0871, Japan.
Int J Mol Sci. 2019 Apr 17;20(8):1893. doi: 10.3390/ijms20081893.
Ubiquitin-like/ubiquitin-associated proteins (UbL-UbA) are a well-studied family of non-proteasomal ubiquitin receptors that are evolutionarily conserved across species. Members of this non-homogenous family facilitate and support proteasomal activity by promoting different effects on proteostasis but exhibit diverse extra-proteasomal activities. Dysfunctional UbL-UbA proteins render cells, particularly neurons, more susceptible to stressors or aging and may cause earlier neurodegeneration. In this review, we summarized the properties and functions of UbL-UbA family members identified to date, with an emphasis on new findings obtained using models showing a direct or indirect role in some neurodegenerative diseases.
泛素样/泛素相关蛋白 (UbL-UbA) 是一类经过充分研究的非蛋白酶体泛素受体家族，在物种间具有高度保守性。该非同源家族的成员通过对蛋白质平衡产生不同的影响来促进蛋白酶体的活性，并表现出多种额外的蛋白酶体活性。功能失调的 UbL-UbA 蛋白使细胞，特别是神经元，更容易受到应激或衰老的影响，并可能导致更早的神经退行性变。在这篇综述中，我们总结了迄今为止鉴定的 UbL-UbA 家族成员的特性和功能，重点介绍了使用显示在某些神经退行性疾病中具有直接或间接作用的模型获得的新发现。
