Fig. 7. C18orf8-, Ccz1- and Mon1A/B- (MCC)-deficient cells accumulate free cholesterol in a swollen lysosomal compartment.

a Filipin staining of wild-type, C18orf8-deficient and complemented C18orf8-deficient cells; or c wild-type, Ccz1- and Mon1A/B-deficient cells. b Filipin (green) co-staining with the LE/Ly markers Rab7 (blue) and LAMP1 (red) in C18orf8-deficient cells. d Theonellamides (TNM) immuno-gold labelling of wild-type and C18orf8-deficient cells, visualised by EM. Multivesicular bodies (MVBs) are indicated by arrows. Note MVBs are markedly enlarged in C18orf8-deficient cells. e Filipin staining of wild-type, C18orf8- and NPC1-deficient cells. All confocal microscopy images are representative of five fields per condition and two independent experiments. Scale bars for confocal microscopy = 10 µm, scale bars for EM = 200 nm.