Therapies in Aicardi-Goutières syndrome

Yanick Crow, A. Vanderver, S. Orcesi, T. W. Kuijpers, G. I. Rice

    Research output: Contribution to journalArticlepeer-review


    Aicardi-Goutières syndrome (AGS) is a genetically determined disorder, affecting most particularly the brain and the skin, characterized by the inappropriate induction of a type I interferon-mediated immune response. In most, but not all, cases the condition is severe, with a high associated morbidity and mortality. A number of important recent advances have helped to elucidate the biology of the AGS-related proteins, thus providing considerable insight into disease pathology. In this study, we outline the clinical phenotype of AGS, paying particular attention to factors relevant to therapeutic intervention. We then discuss the pathogenesis of AGS from a molecular and cell biology perspective. Finally, we suggest possible treatment strategies in light of these emerging insights. © 2013 British Society for Immunology.
    Original languageEnglish
    Pages (from-to)1-8
    Number of pages7
    JournalClinical and experimental immunology
    Issue number1
    Publication statusPublished - Jan 2014


    • Aicardi-Goutières syndrome
    • Anti-interferon antibodies
    • Interferonopathy
    • Reverse transcriptase inhibitors
    • Type I interferon


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