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Fig. 4 | Journal of Neuroinflammation

Fig. 4

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. 4

Distribution of ISG expression in Adar D1113H mutant mouse brain determined by RNA in situ hybridization (ISH). A Brains of WT and Adar D1113H mutant mice photographed at 8-week-old are shown. An apparent difference in gross morphology was not observed. B and C Formalin fixed paraffin embedded (FFPE) sections of 8-week-old mice were stained with hematoxylin and eosin (H&E) (left) or ISH for ISG-15 (middle) or CXCL10 (right). B Cortical sections (S = pial surface, CTX = cortex) show no evidence of inflammatory infiltrate on H&E stain in WT or mutant mice. ISH in mutant mice shows strong labeling of cortical neurons for ISG-15 (middle) and microglia for CXCL10 (right) in mutant but not WT mice. C Deep gray matter sections (V = lateral ventricle, CP = choroid plexus) show no evidence of inflammatory infiltrate on H&E stain in WT or mutant mice. ISH in mutant mice shows strong labeling of cortical neurons and ependyma for ISG-15 (middle) and microglia and ependyma for CXCL10 (right) in mutant, but not WT mice. Bar = 50 microns

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