MCOLN1 and Fabry disease: Pharmacological and genetic activation of TRPML1 ameliorates NPC1‐associated lactosylceramide (LacCer) trafficking defects and cholesterol accumulation (Shen et al, 2012), while activation of the lysosomal big conductance Ca2+‐activated potassium (BK) channel TRPML1 dependently rescues aberrant lysosomal storage in NPA and Fabry disease (Zhong et al, 2016).