KLHL3 and pseudohypoaldosteronism type 2: In addition, the Mendelian disease known as familial hyperkalemic hypertension (FHH) or pseudohypoaldosteronism type II is caused by mutations in two different serine/threonine kinases, WNK1 and WNK4, and in two genes encoding the proteins KLHL3 and CUL3 that form a ring-type E3-ubiquitin ligase that targets WNK kinases for ubiquitylation and degradation [10–12].