Fig. 6

Enlarged endocytic compartments present in LITAF KO or FIG4 KO fibroblasts can be rescued by incubation of cells with the mucolipin activator, ML-SA1. a Immunofluorescence of LITAF KO fibroblasts display enlarged lysosomes, shown by LAMP1 fluorescence that can be reduced by incubation with the TRPML1 agonist, ML-SA1 (40 μM, 36 h). b Quantification of control, LITAF KO or Fig. 4 KO fibroblasts with LAMP1 organelles larger than 2um, with and without incubation with ML-SA1 (40 μM, 36 h). 3 independent experiments were performed, and from each experiment at least 20 cells were quantified. Data shown represents mean and S.E.M. Two-tailed, unpaired T-tests were used to analyse the statistical differences −/+ ML-SA1. *p < 0.05, **p < 0.01, ***p < 0.001. Total number of cells analysed; L261−ML-SA1, 106; L261+ML-SA1, 102; LITAF KO−ML-SA1, 96; LITAF KO+ML-SA1, 98; FIG4 KO–ML-SA1, 79; FIG4 KO + ML-SA1, 83). P values (L261; 0.8519, LITAFKO; 0.04676, FIG4 KO; 0.006978). c Conventional electron microscopy of LITAF KO fibroblasts without and with incubation with the TRPM1 agonist, ML-SA1 (40 μM, 36 h)