The phenotype and elevated cytokine expression levels observed in Trex1−/− knockout mice reflect the clinical presentation of some human autoimmune disorders—notably AGS and Chilblain lupus where mutations within human TREX1 render the primary cellular DNA 3′-to-5′ exonuclease non-functional32, 33. The gene discussed is TREX1; the disease is Aicardi-Goutieres syndrome.