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Fig. 1 | Orphanet Journal of Rare Diseases

Fig. 1

From: Coenzyme Q10 partially restores pathological alterations in a macrophage model of Gaucher disease

Fig. 1

Establishing a chemically-induced Gaucher macrophage model. a THP-1 macrophages were cultured in the presence or absence of CBE (2,5 mM), GlcCer (200 μM) and CBE + GlcCer (2,5 mM + 200 μM) for 72 h. Cells were fixed and immunostained with anti-GlcCer and anti-LAMP-1 (Lysosomal marker) and examined by fluorescence microscopy. Lysosomal marker, LAMP-1, or GlcCer were visualized as red or green, respectively. Colocalization of GlcCer signal with LAMP1 indicates GlcCer lysosomal accumulation b Quantification of GlcCer/LAMP-1 puncta in control and macrophages incubated with CBE, GlcCer and CBE + GlcCer (n = 100 cells). Data represent the mean ± SD of three separate experiments. a p < 0.05 between CBE treatment and control cells. b p < 0.05 between GlcCer supplementation and control cells. c p < 0.05 between CBE + GlcCer combined treatment and CBE or GlcCer treatment. c Representative images of Hematoxylin and eosin staining of chemically-induced Gaucher macrophages

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