Novel interferonopathies associated with mutations in RIG-I like receptors

Type I interferons (IFN) belong to the cytokine family with antiviral function and are involved in regulatory effects on the immune response [1,2]. Secreted type I IFN proteins are synthesised by almost all cell types and mediate immune response by binding to the type I IFN receptor composed of the two chains: IFNAR1 and IFNAR2 [3]. Studies in rats in the early 80 ’s of the last century suggested that elevated IFN levels are detrimental for the mammalian organism [4]. In 1988, Pierre Lebon and colleagues detected high levels of IFN in the cerebrospinal fluid (CSF) from patients with Aicardi-Goutières syndrome (AGS), a rare autoimmune disorder [5].

Source: Cytokine and Growth Factor Reviews - March 10, 2016 Category: Molecular Biology Authors: Insa Buers, Yvonne Nitschke, Frank Rutsch Tags: Survey Source Type: research