Figure 3.

Rosa canina methanol extract (RCME) treatment enhanced the trafficking of NPC1 protein between the ER and the Golgi in a mutation-dependent manner. The fibroblasts derived from a healthy donor (WT), patient 1 (P1), patient 2 (P2), patient 3 (P3), and patient 4 (P4) were treated for 24 h with RCME, miglustat (Mig) or RCME + Mig. The cellular lysates were immunoprecipitated with anti-NPC1 and further treated or not treated with endo H. The immunoprecipitants were subjected to SDS-PAGE and Western blot for NPC1 analysis (A). The ratio of the complex (NPC-c) versus mannose-rich (NPC-h) forms of the NPC1 protein were depicted and the values were normalized to the untreated group (B). Ordinary one-way ANOVA, ns p > 0.05, * p < 0.05, ** p < 0.01 and *** p < 0.001 versus untreated group, S.E.M., and n = 3.