Ubiquitin specific protease (USP)-13 is a de-ubiquitinase member of the cysteine-dependent protease superfamily that cleaves ubiquitin off protein substrates to reverse ubiquitin-mediated protein degradation. Several findings implicate USPs in neurodegeneration. Ubiquitin targets proteins to major degradation pathways, including the proteasome and the lysosome. In melanoma cells, USP13 regulates the degradation of several proteins primarily via ubiquitination and de-ubiquitination. However, the significance of USP13 in regulating protein clearance in neurodegeneration is largely unknown. This mini-review summarizes the most recent evidence pertaining to the role of USP13 in protein clearance via autophagy and the proteasome in neurodegenerative diseases.

泛素特异性蛋白酶（USP）-13是半胱氨酸依赖性蛋白酶超家族的去泛素酶成员，其将泛素从蛋白质底物上裂解下来，以逆转泛素介导的蛋白质降解。一些发现暗示USPs在神经变性中。泛素将蛋白质靶向主要的降解途径，包括蛋白酶体和溶酶体。在黑素瘤细胞中，USP13主要通过泛素化和去泛素化来调节几种蛋白质的降解。但是，USP13在调节神经变性中蛋白质清除中的重要性尚不清楚。这份小型综述总结了有关USP13在神经退行性疾病中通过自噬和蛋白酶体清除蛋白质中的作用的最新证据。