Fig. 5: Correction of storage material and myelin turnover defects in CLN3-deficient microglia using small molecules targeting autophagy/lysosomal function and cholesterol.
From: Loss of CLN3 in microglia leads to impaired lipid metabolism and myelin turnover
a–j Representative micrographs of wild-type and Cln3∆ex7/8/∆ex7/8 microglia, treated with small molecules or DMSO vehicle, as indicated, and subsequently labeled with LipiDyeII or Filipin, 48 h post-myelin washout. k–o For autofluorescence, microglia were unchallenged (no myelin) and unlabeled. Scale bars = 25 μm. p Bar graph shows quantification of lipid droplet number (N = 200 cells/genotype or treatment, from 3 independent experiments). r Bar graph shows quantification of average Filipin intensity (Mean intensities of 240 cells/genotype or treatment, from 3 independent experiments). s Bar graphs show quantification of average autofluorescence intensity (Mean intensities of 180 cells/genotype or treatment, from 3 independent experiments). Data are shown as mean ± s.d.; ns: non-significant, *P ≤ 0.05, **P ≤ 0.01, ***P ≤ 0.001, ****P ≤ 0.0001, One-way ANOVA followed by Tukey’s multiple comparisons test was used.
