The tumor-necrosis-factor receptor-associated periodic syndrome: new mutations in TNFRSF1A, ancestral origins, genotype-phenotype studies, and evidence for further genetic heterogeneity of periodic fevers

Aksentijevich I, Galon J, Soares M, Mansfield E, Hull K, Oh HH, Goldbach-Mansky R, Dean J, Athreya B, Reginato AJ, Henrickson M, Pons-Estel B, O'Shea JJ, Kastner DL

Am J Hum Genet · 2001

Grade Acohort

Key Findings

  • Identified new TNFRSF1A mutations and performed genotype-phenotype analysis
  • Cysteine mutations: 93% penetrance, 24% amyloidosis risk
  • Non-cysteine mutations: 82% penetrance, 2% amyloidosis risk

Referenced in (1 disease)

ID: pmid-11443543DOI: 10.1086/321976PMID: 11443543