Interestingly, KCa3.1-functions have been suggested to be compromised in Fabry disease (FD; Choi et al., 2014, 2015; Choi and Park, 2016), a X-linked lysosomal storage disorder (LSD), in which defective lysosomal targeting of mutated α-galactosidase A encoded by the GLA gene causes globotriaosyl-ceramide (Gb3) accumulation. Here, KCNN4 is linked to Fabry disease.