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Furthermore, an autosomal recessive deficiency in the intracellular protein UNC-93B, as well as different heterozygous mutations in TBK1, the TNFR-associated factor 3 (TRAF3), IFN regulatory factor 3 (IRF3), TYK2, MAVS, TRIF and STAT have been reported to act as factors involved in TLR3 signaling and the activation of IFN responses, and are classified as genetic etiologic mutations associated to HSE (Casrouge et al., 2010