Role of Interferon‐γ–Producing Th1 Cells in a Murine Model of Type I Interferon–Independent Autoinflammation Resulting From <scp>DN</scp>ase <scp>II</scp> Deficiency

Abstract

Dnase2^-/- × Ifnar^-/- DKO mice may be a valid model for exploring the innate and adaptive immune mechanisms responsible for the autoinflammation similar to that seen in DNASE2-hypomorphic patients. In this murine model, IFNγ is required for T cell activation and the development of clinical manifestations. The role of IFNγ in DNASE2-deficient patient populations remains to be determined, but the ability of Dnase2^-/- mouse T cells to transfer disease to Rag1^-/- mice suggests that T cells may be a relevant therapeutic target in patients with IFN-related systemic autoinflammatory diseases.