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Trends Immunol. 2004 Sep;25(9):457-60.

A common pathway in periodic fever syndromes.

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Department of Diabetes and Metabolic Medicine, Unit of Molecular Medicine, Barts and the London, Queen Mary's School of Medicine and Dentistry, University of London, London, UK.


Familial Mediterranean fever (FMF) is an autosomal recessive disease due to mutations in pyrin, which normally inhibits pro-interleukin-1beta (IL-1beta) cytokine processing to the active form. A novel role for pyrin has been proposed by Shoham et al., who studied patients with an autosomal dominant disease called pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome. They demonstrated an interaction between pyrin and proline serine threonine phosphatase-interacting protein 1 (PSTPIP1), the protein involved in PAPA, and thus revealed a biochemical pathway common to both FMF and PAPA.

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