Author(s): Doherty TA, Brydges SD, Hoffman HM
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Abstract Autoinflammatory syndromes are a clinically heterogeneous collection of diseases characterized by dysregulation of the innate immune system. The hereditary recurrent fever disorders were the first to be defined as autoinflammatory. Several of the responsible genes are now known to encode proteins forming multimeric complexes called inflammasomes, which are intracellular "danger sensors" that respond to a variety of different signals by activating caspase-1, responsible for cleavage and subsequent release of bioactive IL-1β. This discovery of the causative link between autoinflammation and IL-1β maturation has led to a significantly improved understanding of the mechanisms of innate immunity, as well as life-altering treatments for patients. Targeting IL-1β for the treatment of autoinflammatory syndromes is an excellent example of the power of translational research. Given the central role of inflammation in many complex multigenic diseases, these treatments may benefit larger numbers of patients in the future. Here, we review current treatment strategies of autoinflammatory diseases with a focus on IL-1 antagonism.
This article was published in J Leukoc Biol
and referenced in Journal of Genetic Syndromes & Gene Therapy