Further studies including a study of PHAII model mice expressing the Cul3 mutant Δ403–459 are required to improve our comprehension of the pathogenic mechanisms of PHAII as related to Cul3 mutations and the regulation of WNK–OSR1/SPAK–NCC signaling by Cul3. Here, STK39 is linked to pseudohypoaldosteronism type 2.