Concerted action of wild-type and mutant TNF receptors enhances inflammation in TNF receptor 1-associated periodic fever syndrome

Simon A, Park H, Maddipati R, Lobito AA, Bulua AC, Jackson AJ, Chae JJ, Ettinger R, de Koning HD, Cruz AC, Kastner DL, Komarow H, Siegel RM

Proc Natl Acad Sci USA · 2010

Grade Acohort

Key Findings

  • WT and mutant TNFR1 act in concert from distinct cellular locations to potentiate inflammation
  • Mutant TNFR1 enhances MAPK activation and cytokine secretion upon LPS stimulation
  • Homozygous mutant mice resemble TNFR1-deficient mice — both alleles needed for disease

Referenced in (1 disease)

ID: pmid-20457915DOI: 10.1073/pnas.0914118107PMID: 20457915