Extended Data Fig. 9: STING signalling activation in Npc1−/− and Npc2−/− cells and mice.
From: Tonic prime-boost of STING signalling mediates Niemann–Pick disease type C
a, Immunoblot analysis of proteins (left) in whole-brain lysates of BALB/c mice of the indicated genotypes (n = 3). b, qRT–PCR analysis of cholesterol-synthesis gene (Hmgcr) expression in the cerebellums of mice of the indicated genotypes (BALB/c) (n = 3). c, Heat map showing the expression of ISGs in BALB/c Npc1+/+, Npc1−/− and Npc2−/− mouse cerebellum (n = 3). The mRNA expression of each ISG was measured by qRT–PCR. d, qRT–PCR analysis of the expression of some ISGs in the cerebellum of mice of the indicated genotypes (BALB/c) (n = 3). e, Immunoblot analysis of the STING signalling cascade. BALB/c Npc1+/+, Npc1−/− and Npc2−/− mouse BMDMs were stimulated with DMXAA (50 μg ml−1) for 0, 2 or 4 h. Phosphorylated and total proteins blotted are shown on the left. f, qRT–PCR analysis of the baseline expression of ISGs in Npc2WT, Npc2KD and Npc2KDSting1KD MEFs (n = 3). g, qRT–PCR analysis of the baseline expression of ISGs in BALB/c Npc2+/+ and Npc2−/− mouse BMDMs that were mock-treated or treated with STING inhibitor C-176 (0.5 μM) overnight (n = 3). b, d, f, g, Data are mean ± s.d. Unpaired two-tailed Student’s t-test. Data are representative of at least two independent experiments.
