Figure 2

Regulation of ENaC by Nedd4-2 and its impairment in Liddle syndrome. In Liddle syndrome, deletion/mutation of the PY motif in βENaC (or γENaC, not shown) impairs the ability of Nedd4-2 to bind (and thus ubiquitylate) ENaC, leading to accumulation of ENaC channels at the plasma membrane and increased channel activity. (Modified with permission from Staub and Rotin).