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Fig. 2 | Journal of Neuroinflammation

Fig. 2

From: An AGS-associated mutation in ADAR1 catalytic domain results in early-onset and MDA5-dependent encephalopathy with IFN pathway activation in the brain

Fig. 2

ISG expression levels were elevated in Adar D1113H mice. A Interferon-stimulated gene (ISG) expression in brain tissues of WT and Adar D1113H mutant mice was quantified using a selected real-time PCR panel covering 11 ISGs as indicated. Within this panel, mRNA levels of all ISGs were significantly increased in Adar D1113H mice, with more than 50-fold increases of ISG-15, IGTP, Ifit-1, and Oasl-2 genes. B ISG expression levels in the brains of Adar D1113H mice were compared with those in the brains of Adar K999N mice. ISG-15, IGTP, IRF7, and Oasl-1 levels were significantly higher in Adar D1113H mice than in Adar K999N mice. C and D ISG expression levels in the livers and spleens of Adar D1113H mice were assessed. In the livers, expression levels of six of seven tested ISGs were higher in Adar D1113H mice than in the controls. Iifit-3b and ISG-15 mRNAs increased by more than 40-fold. In the spleen, expressions of five of seven tested ISGs increased by 3- to 6-fold. E In Adar D1113H mice, ISG expressions in the brain were compared to those in the liver and spleen. All seven tested ISGs were expressed in the brains at a significantly higher level than in the spleens, and five of seven tested ISGs were higher than in the livers. Only Rsad-2 and Ifit-3b were expressed at higher levels in the liver than in the brains. The gene expression levels were calculated using ΔΔt method with reference to the average of GAPDH and HRTP. Increases were expressed as fold changes relevant to levels in wild type mice. Pairwise comparisons (AD) were analyzed using Mann–Whitney U test (AD) and three-way comparison by one-way ANOVA with Tukey adjustment for multiple comparisons. n = 5 (wild type and Adar D1113H group), *P < 0.05, **P < 0.01, ***P < 0.001, ****P < 0.0001

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