Table 1.

Effect of ADAR1 mutations on cells of the immune system.

Cell Type/Organ	Mouse/ Human	Mutation/Gene Deficiency	Functional Effects	Reference
Single positive thymocytes	Mouse	Adar1 deletion in CD4+ T cells	Impaired T cell maturation, excessive ISG expression, defective TCR signaling, induction of autoimmunity (spontaneous colitis)	[54]
Primary CD4+ T lymphocytes	Human	Adar1-deficient AGS patients	Upregulated ISG expression, resistance to HIV-1 infection	[7,56]
mTECs	Mouse	None	Demonstration of high rates of editing events, acquisition of self-tolerance against edited-self, prevention of autoimmunity	[57]
B lymphocytes	Mouse	Adar1 deletion in CD19+ B cells	Impaired B cell maturation, reduced numbers of peripheral blood and splenic B cells, enhanced excessive ISG expression and apoptosis	[9]
Primary macrophages	Human	siRNA ADAR1 silencing	Enhanced production of IFN-I, chemokines and cytokines, inhibition of HIV-1 infection in bystander cells	[59]
DCs	Mouse	Adar1 deletion in CD11c+ cells	Peripheral systemic loss of CD103+/CD8+ DCs, reduced number and dysfunction of AM, loss of RNA editing in AM	[8]

Abbreviations: mTECs, medullary thymic epithelial cells; DCs, dendritic cells; AM, alveolar macrophages.