Dynein-mediated mistrafficking of nephrin has been found as a pathophysiology of progressive human podocytopathies,4,17 especially FSGS caused by the R218Q mutation in the INF2 gene.4 In cultured R218Q KI podocytes, we found an accelerated degradation of nephrin, which could be prevented by bortezomib (a proteasome inhibitor) and Ciliobrevin D (a dynein inhibitor). Here, INF2 is linked to focal segmental glomerulosclerosis.