The identification of missense mutations in TRPC6 (P112Q, R895C, and E897K) that increase TRPC6 activity in recombinant expression systems provided the molecular basis for linking TRPC6 mutations to familial FSGS (Winn et al. 2005; Reiser et al. 2005; Riehle et al. 2016). Here, TRPC6 is linked to focal segmental glomerulosclerosis.