Importantly, it also suggests that reversing dysregulations in vesicular pH—for instance, by using Na+/H+ ionophores (e.g., monensin) that mimic NHE6 function (Mollenhauer et al., 1990; Prasad and Rao, 2015) or by employing protonation agents (e.g., chloroquine) that alkalinize endosomal pH (Akpovwa, 2016; Al-Bari, 2017)—may prove to be beneficial therapeutic strategies to treat CS patients (Figure 3). This evidence concerns the gene SLC9A6 and Cowden syndrome 1.