Activated STING in the thymic epithelium alters T cell development and selection leading to autoimmunity.

ABSTRACT: Coatomer protein complex subunit α (COPA) syndrome is a monogenic disorder of immune dysregulation that leads to interstitial lung disease and high-titer autoantibodies. Constitutive activation of the innate immune molecule stimulator of interferon genes (STING) is centrally involved in disease. However, the mechanisms by which STING results in autoimmunity are not well understood in COPA syndrome and other STING-associated diseases. Prior studies showed a cell autonomous role for STING in thymocyte development. Single-cell data of human thymus demonstrated that STING is highly expressed in medullary thymic epithelial cells (mTECs) and at levels much greater than in T cells. Here, we show that in certain contexts, activated STING exerts a functional role in the thymic epithelium to alter t

SUBMITTER: Deng Z 

PROVIDER: S-EPMC12404769 | biostudies-literature | 2025 Sep

REPOSITORIES: biostudies-literature