As brutally demonstrated by the COVID-19 pandemic, an effective immune system is
essential for survival. Developed over evolutionary time, viral nucleic acid detection is a …

Mounting evidence suggests that DNA damage plays a central role in aging. Multiple tiers of
defense have evolved to reduce the accumulation of DNA damage, including reducing …

CRISPR-Cas9 is a versatile genome editing technology for studying the functions of genetic
elements. To broadly enable the application of Cas9 in vivo, we established a Cre …

Aicardi–Goutières syndrome is an inflammatory disease occurring due to mutations in any of
TREX1, RNASEH2A, RNASEH2B, RNASEH2C, SAMHD1, ADAR or IFIH1. We report on 374 …

Objective Autoinflammatory type I interferonopathies, chronic atypical neutrophilic
dermatosis with lipodystrophy and elevated temperature/proteasome-associated …

Aicardi-Goutières syndrome (AGS) is a rare, genetically determined early-onset progressive
encephalopathy. To date, mutations in six genes have been identified as etiologic for AGS …

Considering that it is a single exon gene encoding a 314 amino acid protein, the genotype-
phenotype landscape of TREX1 is remarkably complex. Here we briefly describe the human …

Adaptive immune responses to Ags released by dying cells play a critical role in the
development of autoimmunity, allograft rejection, and spontaneous as well as therapy …

Highlights•Self-DNA activates STING regulated autoinflammatory responses.•Nucleases
such as DNaseII and III prevent inflammation by degrading STING activators.•Gain of …

Aicardi-Goutières syndrome (AGS) is a genetically determined encephalopathy
demonstrating phenotypic overlap both with the sequelae of congenital infection and with …