. 2022 Jun 22;18(9):558–572. doi: 10.1038/s41581-022-00589-6

Table 1.

Systemic diseases associated with cGAS–STING activation in humans or mice

Disease names	Mouse models	Human diseases
AGS	Activation of cGAS–STING by loss of function mutations of DNases or nucleic acid metabolizing enzymes^43,65,86,87	Mutations in TREX1, RNASEH and other nucleic acid processing enzymes lead to the activation of cGAS and the production of IFNβ^83,84
SAVI	Gain-of-function mutations in Sting1 lead to SAVI-like diseases^90–92	Gain-of-function mutations in STING1 result in a systemic inflammatory disease with vasculitis and pulmonary fibrosis^88,89
SLE	cGAS–STING is activated in the lupus-prone TREX1 D18N mutant⁹⁵ and Fcgr2b-knockout mice⁹⁶	TREX1 heterozygous mutations associated with familial chilblain lupus¹⁰⁰ cGAMP expression in PBMCs of 15% of patients with SLE and in microvesicles from SLE blood cells^102,103 Mitochondria-rich red blood cells activate IFN-I through cGAS–STING in lupus monocytes¹⁰⁸
Ageing and senescence	Activation of cGAS–STING-triggered immune senescence^123,126,127 cGAS drives non-canonical inflammasome activation in age-related macular degeneration¹⁸⁰	Activation of cGAS–STING triggered immune senescence^123,126,127 cGAS drives non-canonical inflammasome activation in age-related macular degeneration¹⁸⁰

AGS, Aicardi–Goutières syndrome; cGAS, cyclic GMP–AMP synthase; IFN-I, type I interferon; PBMCs, peripheral blood mononuclear cells; SAVI, STING-associated vasculopathy with onset in infancy; SLE, systemic lupus erythematosus; STING, stimulator of interferon genes.