CUL3 and pseudohypoaldosteronism type 2: We clarified that WNK4 is a substrate of the KLHL3–Cullin-3 E3 ligase complex, and that the impaired ubiquitination of WNK4 protein and, subsequent, increase of WNK4 protein abundance in DCT would represent a common molecular pathogenesis of PHAII caused by mutations in WNK4, KLHL3 or Cullin-3 [16].