What is tumor necrosis factor receptor periodic syndrome?
Tumor Necrosis Factor Receptor Associated Periodic Syndrome (TRAPS) is a rare genetic disease with episodes of recurrent fever; abdominal, chest, and muscle pain; red and swollen eyes; and a typical rash lasting for more than one week. Most patients present in the first decade of life.
What does TRAF6 stand for?
Aliases. TRAF6, MGC:3310, RNF85, TNF receptor associated factor 6.
How many TNF receptors are there?
TNF receptors are an family of proteins that consist of, to date, at least 27 members characterized by their repeated cysteine-rich extracellular sequence homology, and include LT receptor, Fas, CD40, the low affinity nerve growth factor receptor, TRAIL receptors, RANK and death or decoy receptors (Darnay & Aggarwal.
Where are TNF receptors located?
plasma membrane
In their active form, the majority of TNF receptors form trimeric complexes in the plasma membrane. Accordingly, most TNF receptors contain transmembrane domains (TMDs), although some can be cleaved into soluble forms (e.g. TNFR1), and some lack a TMD entirely (e.g. DcR3).
What does tumor necrosis factor do?
Tumor necrosis factor (TNF) is a multifunctional cytokine that plays important roles in diverse cellular events such as cell survival, proliferation, differentiation, and death. As a pro-inflammatory cytokine, TNF is secreted by inflammatory cells, which may be involved in inflammation-associated carcinogenesis.
Can PFAPA go away and come back?
Episodes of PFAPA usually last a few days to a week. These episodes may come back every few weeks. Between these episodes, children are completely well.
What cells have TNF receptors?
TNF is a transmembrane 26 KDa protein expressed by activated monocytes/macrophages (including central nervous system (CNS) microglia), activated NK and T cells, but also by a diverse array of non-immune cells such as endothelial cells and fibroblasts [27], [28].
What does an elevated tumor necrosis factor mean?
If you have a severe bacterial infection like pneumonia, high levels of tumor necrosis factor are a sign of inflammation that’s helping you heal.
Is PFAPA an autoimmune disorder?
PFAPA is an autoinflammatory, not autoimmune, disease and does not have a known trigger. Due to inappropriate activation and regulation of antigen-independent inflammation (innate immunity).
Is PFAPA serious?
PFAPA doesn’t cause severe symptoms or complications. Because of this, some healthcare providers may advise against any treatment. Even without treatment, PFAPA usually stops sometime after age 10. In rare cases, a child may still have symptoms into adulthood, though usually less often.
What does TNF stimulate?
TNF promotes the inflammatory activity of macrophages but also controls macrophage survival and death. TNF exerts its activities by stimulation of two different types of receptors, TNF receptor-1 (TNFR1) and TNFR2, which are both expressed by macrophages.
Why is it called tumor necrosis factor?
The designation TNF or tumor necrosis factor reflects the original discovery in the 1970s of a cytotoxic substance produced by immune cells stimulated by endotoxin.
What causes tumor necrosis factor?
A protein made by white blood cells in response to an antigen (substance that causes the immune system to make a specific immune response) or infection.
What triggers PFAPA?
Is PFAPA life long?
PFAPA does not cause severe symptoms or long-term complications. PFAPA syndrome usually goes away on its own in the second decade of life.
What kind of doctor treats PFAPA?
Usually, a rheumatology clinic will have a team of doctors and nurses who have a lot of experience treating children with PFAPA.