COPA and autoimmune interstitial lung disease-arthritis syndrome: The heterozygous missense mutations in coatomer protein subunit alpha (COPA, a subunit of coat protein complex-I or COP-I that mediates Golgi to ER transport) cause COPA syndrome (an autosomal dominant autoimmune dysregulatory disease, involving lungs and joints) overlapping clinically with the higher type 1 IFN levels due to the gain of function in the STING, even in the absence of its ligand (39, 40).