Topic: Rnaset2-/- mice

We have recently established Rnaset2-/- mice as a model system to improve the pathophysiological understanding of infantile-onset RNaseT2-deficient leukoencephalopathies and type I interferonopathies. Rnaset2-/- mice were viable and healthy at birth, but displayed upregulation of type I interferon-stimulated genes in all organs examined. At 20 weeks of age, RNaseT2-deficient animals developed IFNAR1-dependent neuroinflammation, characterized primarily by the presence of CD8 T cells and inflammatory monocytes. Single nuclei transcriptome analyses revealed homeostatic dysfunction in glial cells

Type I interferon (IFN-I) is a pivotal cytokine of the innate immune response, playing a significant role in the pathophysiology of inflammation-induced damage in the central nervous system (CNS). Enhanced, constitutive IFN-I signaling is a defining feature of a number of Mendelian inborn errors of immunity, which are coined type I interferonopathies. However, understanding the pathophysiology of these disorders is hindered by the inability of numerous animal models to replicate the neurological phenotype essential for these diseases. Fortunately, we have recently generated and characterized t