As shown in Fig. 7A, human skin fibroblasts from Fabry disease patients (GM00107)28 displayed a much higher level of lipofuscin autofluoresence compared to wild type human fibroblasts, and this was decreased by activating TRPML1 with ML-SA1 (15 μM, 16 h), and by activating BK with NS1619 (15 μM, 16 h) or NS11021 (3 μM, 16 h) (Fig. 7 A–C), respectively. Here, MCOLN1 is linked to Fabry disease.