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Clinical Implications of Basic Research
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Volume 355:730-732 August 17, 2006 Number 7
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The Inflammasome — A Linebacker of Innate Defense
Joost P.H. Drenth, M.D., Ph.D., and Jos W.M. van der Meer, M.D., Ph.D.

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Why study hereditary periodic-fever syndromes, which affect fewer than 500 patients worldwide? The discovery of cryopyrin provides an answer. The story begins in 2001, when investigators showed that mutations in the cold-induced autoinflammatory syndrome 1 (CIAS1) gene, which encodes cryopyrin, causes two hereditary periodic-fever disorders: the Muckle–Wells syndrome and the familial cold autoinflammatory syndrome.1 The following year, a third periodic-fever syndrome (also referred to as neonatal-onset multisystem inflammatory disease or the chronic infantile neurologic, cutaneous, articular syndrome) was found to be caused by mutant cryopyrin.2,3 Three articles recently published in Nature4,5,6 now suggest that by discovering cryopyrin, the . . . [Full Text of this Article]


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From the Departments of Gastroenterology, Hepatology, and General Internal Medicine, Radboud University Medical Center, Nijmegen, the Netherlands.


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