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. 2024 Jan 29;27(3):108959. doi: 10.1016/j.isci.2024.108959

Figure 9.

Figure 9

AMPK inhibition rescues lysosomal defects and HS accumulation in MPS IIIB cell model systems

(A) SK-NBE (CTRL) and ΔNAGLU clones were grown on coverslips and treated with SBI-0206965, a specific AMPK inhibitor (AMPKi), and 5-aminoimidazole-4-carboxamide ribonucleoside (AICAr), a AMPK allosteric activator (AMPKa), for 24 h before being processed for indirect immunofluorescence. The lysosomal marker LAMP1 (green) and heparan sulfate (pink) proteins were revealed by using specific antibodies. Nuclei (blue) were decorated by DAPI staining. Single focal sections are shown. Images are representative of three independent experiments made in triplicates. Scale bar: 50 μm.

(B) MPS IIIB fibroblasts were treated with SBI-0206965 and processed as in A. Single focal sections are shown. Images are representative of three independent experiments made in triplicates. Scale bar: 50 μm. The histograms on the right for (A) and (B) show the quantification relative to the percentage of cells with pathological enlarged lysosomes (green bars) and HS means fluorescence intensity (pink bars). Means ± SEM were obtained from three independent experiments. ∗∗∗ p value <0.001. ns = not significant.