Article
Molecular mechanisms in genetically defined autoinflammatory diseases: disorders of amplified danger signaling.
Annual review of immunology - 1 Jan 2015
de Jesus Adriana Almeida, Canna Scott W, Liu Yin, Goldbach-Mansky Raphaela
Abstract excerpt
Patients with autoinflammatory diseases present with noninfectious fever flares and systemic and/or disease-specific organ inflammation. Their excessive proinflammatory cytokine and chemokine responses can be life threatening and lead to organ damage over time. Studying such patients has revealed genetic defects that have helped unravel key innate immune pathways, including excessive IL-1 signaling, constitutive...
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