WNK2 and Netherton syndrome: Increased Ras/MAPK signaling seems to play a major role in the pathogenesis of NS-related defects, since several studies demonstrated that the inhibition of this signaling cascade by pharmacological inhibitors of the mitogen-activated protein kinase kinase (shortly called MEK) was able to fully or partially rescue cardiac [14,15] and craniofacial malformations [16], as well as defects in growth hormone release, which likely contribute to growth retardation seen in patients with NS [13].