Figure 2. IFG increases the lysosomal pool of L444P GCase in Gaucher patient-derived fibroblasts.
Fibroblasts derived from healthy volunteers (WT; CRL2097) and Gaucher patients homozygous for the N370S (DMN89.45) or L444P (GM07968, GM00877, GM10915) mutant forms of GCase were incubated in the absence or presence of 100 μM IFG tartrate for 14 days. GCase (green) and the lysosomal marker LAMP-1 (red) were visualized by confocal microscopy after indirect immunofluorescence staining as described in ‘Materials and Methods’. In the merged images, yellow denotes co-localization of the two proteins, indicative of their lysosomal localization. Nuclei are stained with DAPI (blue). IFG treatment increased the lysosomal pool of GCase in wild-type as well as N370S and L444P GCase fibroblasts (as shown by the increased amount of yellow in the merged images). Representative cells are shown to demonstrate the degree of co-localized GCase and LAMP-1. Magnification: 63x.