Notable proteins from this family include Keap1 (KLHL19), an electrophile-sensing regulator of Nrf2 [10]–[12], KLHL9, which is associated with an autosomal distal myopathy [13], KLHL12, a regulator of the dopamine D4 receptor [14] Dishevelled [15], [16] and CPOII coat function [17], KLHL20, a regulator of hypoxia-inducible factors [18], [19], and KLHL3, a regulator of hypertension with mutations identified in pseudohypoaldosteronism type II (PHAII) [20]–[22]. This evidence concerns the gene KLHL3 and pseudohypoaldosteronism type 2.