Failure of anti-TNF therapy in TNF Receptor 1-Associated Periodic Syndrome (TRAPS)
نویسندگان
چکیده
منابع مشابه
Comment on: Failure of anti-TNF therapy in TNF receptor 1-associated periodic syndrome (TRAPS).
There was no history of ischaemic heart disease, but a routine ECG performed in April 2006 showed first-degree heart block, at a rate of 70 beats/min. Patients having infliximab in our unit are admitted overnight for the first three infusions. The first infusion was uneventful at week 0. Following the second infusion 2 weeks later, she then gave a history of dry cough for 3–4 months. A chest X-...
متن کاملFalling into TRAPS – receptor misfolding in the TNF receptor 1-associated periodic fever syndrome
TNF receptor-associated periodic syndrome (TRAPS) is a dominantly inherited disease caused by missense mutations in the TNF receptor 1 (TNFR1) gene. Patients suffer from periodic bouts of severe abdominal pain, localised inflammation, migratory rashes, and fever. More than 40 individual mutations have been identified, all of which occur in the extracellular domain of TNFR1. In the present revie...
متن کاملA pro-inflammatory signalome is constitutively activated by C33Y mutant TNF receptor 1 in TNF receptor-associated periodic syndrome (TRAPS)
Mutations in TNFRSF1A encoding TNF receptor 1 (TNFR1) cause the autosomal dominant TNF receptor-associated periodic syndrome (TRAPS): a systemic autoinflammatory disorder. Misfolding, intracellular aggregation, and ligand-independent signaling by mutant TNFR1 are central to disease pathophysiology. Our aim was to understand the extent of signaling pathway perturbation in TRAPS. A prototypic mut...
متن کاملCharacterization of the TNFR1-d2 protein: Implication in TNF receptor associated periodic syndrome (TRAPS)?
Introduction Binding of TNF to TNF receptor 1 (TNFR1) induces both the survival pathway by activation of the NF-kB transcription factor, and the death pathway by apoptosis. Mutations in the TNFR1 gene (TNFSFR1A) are responsible for the auto-inflammatory disease TRAPS, a dominantly inherited hereditary recurrent fever. Various defects such as defective TNFR1 receptor shedding, protein misfolding...
متن کاملConcerted action of wild-type and mutant TNF receptors enhances inflammation in TNF receptor 1-associated periodic fever syndrome.
TNF, acting through p55 tumor necrosis factor receptor 1 (TNFR1), contributes to the pathogenesis of many inflammatory diseases. TNFR-associated periodic syndrome (TRAPS, OMIM 142680) is an autosomal dominant autoinflammatory disorder characterized by prolonged attacks of fevers, peritonitis, and soft tissue inflammation. TRAPS is caused by missense mutations in the extracellular domain of TNFR...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: Rheumatology
سال: 2007
ISSN: 1462-0324,1462-0332
DOI: 10.1093/rheumatology/kel298