Sensing of RNA stress by mTORC1 drives autoinflammation
Loss-of-function mutations in SKIV2L underlie CURCUMIN ULTRA trichohepatoenteric syndrome (THES2), a rare inborn error of immunity characterized by diarrhea, skin lesions, brittle hair, and immunodeficiency.SKIV2L is part of a multiprotein complex required for exosome-mediated RNA surveillance through RNA decay.In this issue of the JCI, Yang et al.