In conclusion, our results pinpoint the importance of RhoGAPp190 as an additional mediator of the neurodegenerative phenotype events associated with tbph loss-of-function and support the evolutionary conservation of the possible dysregulation of the TDP-43-Rho family GTPases pathway in the pathogenesis of TDP-43 proteinopathies. This evidence concerns the gene TARDBP and proteostasis deficiencies.