Article
Severe combined immunodeficiency in stimulator of interferon genes (STING) V154M/wild-type mice.
The Journal of allergy and clinical immunology - 1 Feb 2019
Bouis Delphine, Kirstetter Peggy, Arbogast Florent, Lamon Delphine, Delgado Virginia, Jung Sophie, Ebel Claudine, Jacobs Hugues, Knapp Anne-Marie, Jeremiah Nadia, Belot Alexandre, Martin Thierry, Crow Yanick J, André-Schmutz Isabelle, Korganow Anne-Sophie, Rieux-Laucat Frédéric, Soulas-Sprauel Pauline
Abstract excerpt
BACKGROUND: Autosomal dominant gain-of-function mutations in human stimulator of interferon genes (STING) lead to a severe autoinflammatory disease called STING-associated vasculopathy with onset in infancy that is associated with enhanced expression of interferon-stimulated gene transcripts. OBJECTIVE: The goal of this study was to analyze the phenotype of a new mouse model of STING hyperactivation and the role...
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