Effect of Heat Shock Proteins on Life Span and Behavioral Functions of Animals with FUS Proteinopathy

—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.