Fig. 5: Aberrant autophagic flux in SMS patient-derived cells. | Cell Death & Disease

Fig. 5: Aberrant autophagic flux in SMS patient-derived cells.

From: Retinoic acid-induced 1 gene haploinsufficiency alters lipid metabolism and causes autophagy defects in Smith-Magenis syndrome

Fig. 5: Aberrant autophagic flux in SMS patient-derived cells.The alternative text for this image may have been generated using AI.

A Immunofluorescence analysis of LC3 levels showing the accumulation of LC3 in SMS cells. Left panel: Representative images of n = 3 biological replicates. Right panel: Quantification of LC3 fluorescence intensity in 150 cells from three biological replicates. B, C Western blot analysis of the LC3 and p62 levels in SMS (RAI1-del1 and RAI1-S399P40fx) cells compared with sibling and CTR (sibling and CTR1) cells. Top panels: Representative images from three biological replicates. Bottom panels: Quantification of Western blots. D Western blotting analysis of the LC3 and p62 levels in SMS (RAI1-del1 and RAI1-S399P40fx) cells compared with sibling and CTR (sibling and CTR1) cells treated with either vehicle or chloroquine (CQ, 40 mM, 24 h). Top panels: Representative images from three biological replicates. Bottom panels: Quantification of Western blots. E LysoTracker staining analysis showing lysosomal accumulation in four SMS and four CTR cell lines. Left panel: Representative images of n = 3 biological replicates. Right panel: Quantification of LysoTracker fluorescence intensity in 100 cells from three biological replicates. Graphs: mean ± SEM, one-way ANOVA + Newman–Keuls post hoc test, *p < 0.05, **p < 0.01, ***p < 0.001.

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