FIGURE 4.

EerI facilitates N370S GC folding, lysosomal trafficking, and activity in GD patient-derived fibroblasts. A, relative N370S GC activities were measured in cells treated with proteostasis regulators (MG-132 (0.2 μm) and celastrol (0.2 μm)) and a range of EerI concentrations for 72 h. Relative GC activities were evaluated as described in the legend to Fig. 2 (p < 0.01 if not specified; *, p < 0.001). Experiments were repeated three times, and data points are reported as mean ± S.D. (error bars). MG, MG-132; Cel, celastrol. Shown are immunofluorescence microscopy images of GC and CNX (an ER marker) (B) and GC and LAMP-1 (a lysosomal marker) (C) in cells treated with EerI (2 μm) and MG-132 (0.2 μm) for 48 h. Colocalization images were analyzed as described in the legend to Fig. 3.