Fig. 1

Analysis of the microglia in Fabry disease and mucolipidosis type IV mice. Mean fluorescence intensity (MFI) of the surface expression of microglial lineage markers a CX3CR1 and CD11b and microglial activation markers. N ≥ 5; *p < 0.05, **p < 0.01 Mann-Whitney U test vs. WT. b CD86 and MHCII. WT, control; FD, 2-month-old Fabry disease; MLIV, 2-, 4-, and 8-month-old mucolipidosis type IV. N ≥ 5; *p < 0.05, **p < 0.01 Mann-Whitney U test vs. WT. c DCFDA analysis of free radical production by 10,000 microglial cells. 1 μM H₂O₂ treatment was used as a positive control for free radical production. N = 5, n ≥ 3; *p < 0.05, **p < 0.01 Mann-Whitney U test vs. WT. d Western blot analysis iNOS, P-ERK1/2, ERK1/2, and HIF1α and β-tubulin on 100,000 microglia lysates. N = 4, *p < 0.05 Mann-Whitney U test. e Basal extracellular acidification rate (ECAR) in mpH.minutes⁻¹ of unbuffered seahorse media by 10,000 cells. N = 5, n ≥ 3; *p < 0.05 Mann-Whitney U test vs. WT