Abstract—It is known that chaperones and, in particular, 70 kDa heat shock proteins (HSP70), are an integral part of cellular proteostasis. To assess the role of HSP70 in the neurodegenerative process, we used a new hybrid strain of mice with co-expression of the aberrant ΔFUS protein (1–359) and human HSP70. The mice of the new hybrid line are characterized by a longer lifespan, as well as an increase in the duration of the symptomatic stage as compared to control animals. In addition, according to the results of physiological tests, it can be concluded that HSP70 has a positive effect on muscle strength at the symptomatic stage of the model disease.

摘要—众所周知，伴侣蛋白，尤其是70 kDa的热休克蛋白（HSP70）是细胞蛋白稳定的组成部分。为了评估HSP70在神经退行性过程中的作用，我们使用了一种新的杂合小鼠品系，其共同表达了异常ΔFUS蛋白（1-359）和人类HSP70。与对照动物相比，新杂种系的小鼠具有更长的寿命以及症状期持续时间更长的特点。另外，根据生理学测试的结果，可以得出结论，HSP70在模型疾病的症状阶段对肌肉力量具有积极作用。