Article
The expanding spectrum of systemic autoinflammatory disorders and their rheumatic manifestations.
Current opinion in rheumatology - 1 Jan 2003
Hull Keith M, Shoham Nitza, Chae Jae Jin, Aksentijevich Ivona, Kastner Daniel L
Abstract excerpt
The authors review the genes, and their respective proteins, responsible for eight autoinflammatory conditions. Familial Mediterranean fever is caused by mutations in pyrin, which is the prototype of a new family of proteins belonging to the death-domain superfamily. This new group of proteins, which regulate apoptosis, inflammation, and cytokine processing, share an approximately 90-amino-acid N-terminal...
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