STING-Associated Vasculopathy with Onset in Infancy — A New Interferonopathy

The past 20 years have seen remarkable progress in our understanding of pathogen recognition by hematopoietic and nonhematopoietic cells. Central to this process is the sensing of microbial components by germline-encoded receptors. In the case of viruses, viral RNA and DNA are chief among the biologic motifs recognized by these receptors.In 2006, it was shown that transfection of DNA into various cell types induced a toll-like receptor (TLR)–independent up-regulation of type 1 interferons.1,2 In this context, the stimulator of interferon genes (STING) protein, a transmembrane protein that is localized to the endoplasmic reticulum, was described in 2008 as . . .