Ontology highlight
ABSTRACT:
SUBMITTER: Piperno GM
PROVIDER: S-EPMC7526445 | biostudies-literature | 2020 Sep
REPOSITORIES: biostudies-literature
Piperno Giulia Maria GM Naseem Asma A Silvestrelli Giulia G Amadio Roberto R Caronni Nicoletta N Cervantes-Luevano Karla Evelia KE Liv Nalan N Klumperman Judith J Colliva Andrea A Ali Hashim H Graziano Francesca F Benaroch Philippe P Haecker Hans H Hanna Richard N RN Benvenuti Federica F
JCI insight 20200903 17
Dysregulated sensing of self-nucleic acid is a leading cause of autoimmunity in multifactorial and monogenic diseases. Mutations in Wiskott-Aldrich syndrome protein (WASp), a key regulator of cytoskeletal dynamics in immune cells, cause autoimmune manifestations and increased production of type I IFNs by innate cells. Here we show that immune complexes of self-DNA and autoantibodies (DNA-ICs) contribute to elevated IFN levels via activation of the cGAS/STING pathway of cytosolic sensing. Mechani ...[more]