Figure 6. Recombinant ERp57 restored PGRN- and ND7-mediated increase of GCase activity and decrease of β-GlcCer accumulation in ERp57 KO L444P fibroblasts.

(A) The GCase activity in ERp57 KO L444P with or without ND7 (5 μg/mL) and PGRN (10 μg/mL), or ND7 and PGRN plus rERp57 (2 μg/mL) for 24 hours. PBS and rErp57 (2 μg/mL) were used as controls. The cell lysate was used to measure the GCase activity, assayed by released 4MU-Glc. (B) β-GlcCer accumulation in ERp57 KO L444P with or without ND7 (5 μg/mL) and PGRN (10 μg/mL), or ND7 and PGRN plus rERp57 (2 μg/mL) for 24 hours, analyzed by immunofluorescence staining with antibody against β-GlcCer. (C) Quantification of mean fluorescence intensity of B. Data are shown as mean ± SD of 3 independent experiments. One-way ANOVA tests. *, p<0.05, **, p<0.01. ns: not significant. Scale bar=100 μm.