Extended Data Fig. 4: Various lysosome storages mutually induce STING signalling.
From: Innate immune sensing of lysosomal dysfunction drives multiple lysosomal storage disorders
(a, b) 24-hour treatment of BafA1 in HeLa cells led to clustering and co-localizing STING with the ERGIC, GM130 (Glogi marker), and LAMP1 (Lysosome marker) (a) and quantification (b). Scale bar, 20 μm. n = 22 cells per group, two-way ANOVA with Bonferroni correction, mean ± SEM. (c) Quantitative real-time RT-PCR indicated that BafA1-induced lysosomal storage promotes IFN-I and ISG mRNA expression, downstream effectors of STING signalling. n = 4 independent samples. (d, e) Immunoblottings and qRT-PCR assays indicated a profound increase of cGAS-STING signalling and ISGs expression in Npc1 KO N2a cells upon treating STING agonist DMXAA. n = 3 independent samples, two-way ANOVA with Bonferroni correction, mean ± SEM. (f) Immunoblottings revealed an enhanced STING signalling upon diABZI treatment without Gla. (g) Increased clustering STING and phosphorylated STING in Gla-/- DRG neurons were detected and quantificated. n = 10 images per group. Unless otherwise specified, error bars in graphs c and g represent mean ± SEM, and P values were calculated by one-way ANOVA with Bonferroni correction. Results in a–g represent three independent experiments.
