KCNMA1 and Fabry disease: A potential link between Fabry pain and Ca2+ levels was already assumed: studies reported alterations of Ca2+-activated ion channel expression in Fabry disease, such as KCa1.1 in patient fibroblasts,54 KCa3.1 in Gb3-treated human umbilical vein endothelial cells and aortic endothelial cells from a Fabry mouse model.55 Further, elevated intracellular Ca2+ concentrations were associated with increased lyso-Gb3 levels, the deacylated derivative of Gb356 in murine DRG neurons leading to pain-related behaviour.10