WNK1 (with no lysine 1) is a member of the WNK family of serine-threonine kinases. The WNK family of kinases has been observed to play a role in several cellular processes which include ion balance, signal transduction, survival, proliferation and organ development. WNK1 appears to be important to ion homeostasis. Mutations in WNK1 cause pseudohypoaldosteronism type II (PHA II), also called Gordon’s syndrome, an autosomal dominant disease characterized by defects in ion transport in the nephron that result in hypertension and hyperkalemia. WNK1 has been reported to regulate sodium and chloride ion transport by influencing the activity of another WNK family member, WNK4. WNKF1 has also been observed to regulate ion homeostasis independent of WNK4.