Our results highlight the importance of receptor complex composition in disease development and suggest that TILRR may provide an easily accessible therapeutic target for subtle site- and signal-specific inhibition of inflammatory activation during development of vascular disease.PerspectivesCOMPETENCY IN MEDICAL KNOWLEDGE: The cytokine IL-1 and its signaling receptor IL-1RI are central regulators of vascular disease. Here, IL1B is linked to glycogen storage disease VI.