Mutations of the PPxY-motif in SCNN1B are linked to Liddle syndrome 1, a hereditary form of hypertension caused by augmented Na2+ transport, and result in the loss of interaction with the HECT type E3 ligases and increased stability of the transmembrane protein (Enslow et al, 2019). The gene discussed is SCNN1B; the disease is Liddle syndrome 1.