Extended Data Fig. 7: STING and IRF3, but not cGAS, are required for Npc1−/− disease pathogenesis. | Nature

Extended Data Fig. 7: STING and IRF3, but not cGAS, are required for Npc1−/− disease pathogenesis.

From: Tonic prime-boost of STING signalling mediates Niemann–Pick disease type C

Extended Data Fig. 7

a, Representative image for the body size of eight-week-old mice. b, Immunoblot analysis of proteins (left) in whole-brain lysates of mice of the indicated genotypes (C57BL/6J; n = 3). c, Heat map showing the expression of ISGs in BMDMs from mice of the indicated genotypes (C57BL/6J; n = 3). The mRNA expression level of each ISG was measured by qRT–PCR. d, Serum cytokine levels in four-week-old mice of the indicated genotypes measured by multiplex ELISA (n = 4). e, Heat map showing the expression of ISGs in Npc1+/+ and Npc1−/− BMDMs that were mock-treated or treated with the STING inhibitor C-176 (0.5 μM) or C-178 (0.5 μM) overnight (n = 2). The mRNA expression of each ISG was measured by qRT–PCR. d, Data are mean ± s.d. Unpaired two-tailed Student’s t-test. Data are representative of at least two independent experiments.

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