Atlas One · Host Biology · Scale 07 — System

Aicardi-Goutières Syndrome

Aicardi-Goutières syndrome (AGS) is a genetic interferonopathy caused by LOF mutations in nucleic acid metabolism genes (TREX1, RNASEH2A/B/C, SAMHD1, ADAR1, IFIH1) → cytosolic nucleic acid accumulation → cGAS-STING activation → chronic IFN-α/β → progressive encephalopathy.

Also known as: AGS, Aicardi-Goutieres syndrome, Cree encephalitis, pseudo-TORCH syndrome, interferonopathy, TREX1 deficiency, RNASEH2 deficiency, SAMHD1 deficiency, ADAR1 deficiency, IFIH1 deficiency, familial chilblain lupus

Scale 07 — SystemScale
draftStatus
2026-06-08Last Reviewed
This is an auto-generated stub page built from atlas entry frontmatter. A richer hand-crafted page is planned. See the atlas source for the full entry including detailed body sections.
aicardi-goutieres-syndrome

Aicardi-Goutières Syndrome

Aicardi-Goutières syndrome (AGS) is a genetic interferonopathy caused by LOF mutations in nucleic acid metabolism genes (TREX1, RNASEH2A/B/C, SAMHD1, ADAR1, IFIH1) → cytosolic nucleic acid accumulation → cGAS-STING activation → chronic IFN-α/β → progressive encephalopathy.

Entry Metadata

FieldValue
IDaicardi-goutieres-syndrome
NameAicardi-Goutières Syndrome
Statusdraft
Last reviewed2026-06-08
Atlas01-human
Scale07-system

Cross-Atlas Connections

connects-toCgas Sting connects-toSystemic Lupus Erythematosus connects-toType I Interferon connects-toMicrocephaly connects-toHiv 1 connects-toNf Kb connects-toMicroglia connects-toJak1 2 connects-toBrain connects-toDermatomyositis connects-toSkin connects-toAstrocyte connects-toNeuron connects-toDendritic Cell connects-toSjogrens Syndrome connects-toMultiple Sclerosis connects-toStat1 connects-toEpilepsy connects-toMavs connects-toNervous System connects-toImmune System connects-toCalcium connects-toT Cytotoxic Cell connects-toWest Nile Virus connects-toRig I connects-toRegulatory T Cell connects-toLiver connects-toOligodendrocyte connects-toPhosphorus connects-toPlatelet connects-toEndothelial Cell connects-toSpleen connects-toSynapse connects-toPhoton connects-toBone Marrow connects-toEye connects-toElectron connects-toHeart connects-toKidney connects-toAntibody connects-toErythrocyte connects-toHepatocyte connects-toMusculoskeletal System connects-toB Cell connects-toThyroid connects-toMacrophage connects-toSystemic Sclerosis connects-toIl 6 connects-toIrf3 connects-toStroke connects-toFibroblast connects-toTnf Alpha connects-toIfn Gamma connects-toSepsis connects-toPulmonary Arterial Hypertension connects-toCkd connects-toAnemia Of Chronic Disease connects-toAspergillus Fumigatus connects-toType 1 Diabetes connects-toNeuropathic Pain connects-toIntegumentary System connects-toDigestive System connects-toRespiratory System connects-toCardiovascular System connects-toEndocrine System connects-toLymphatic System connects-toRenal System connects-toReproductive System connects-toToxoplasma Gondii connects-toCovid 19 Disease connects-toNeutrophil connects-toZika Virus connects-toTargeted Therapy connects-toHerpesvirus connects-toArterial Wall connects-toGlomerulus connects-toHepatic Lobule connects-toPsoriasis connects-toRheumatoid Arthritis connects-toGerminal Center connects-toCortical Bone connects-toAxonal Transport connects-toCll connects-toAlzheimers Disease connects-toTbk1 connects-toIl 1B connects-toNlrp3 Inflammasome connects-toCcl2 connects-toPerforin connects-toAutophagy connects-toCaspase 3 connects-toVegf connects-toMhc Class Ii connects-toCalcium connects-toComplement C3 connects-toS100A8 A9 connects-toMtor connects-toStat3 connects-toGlutamate connects-toBcl 2 connects-toMyd88 connects-toEndothelin 1 connects-toGalectin 3 connects-toAkt connects-toErk1 2 connects-toPdgf connects-toGsk 3B connects-toSmad4 connects-toFoxo connects-toHif 1Alpha connects-toComplement C5 connects-toPik3Ca connects-toSrc Kinase connects-toAmpk connects-toDnmt3A connects-toCcr5 connects-toMdm2 connects-toCxcl12 connects-toIl 33 connects-toIl 10 connects-toIl 17A connects-toArid1A connects-toCalcineurin connects-toNitric Oxide connects-toImmunoglobulin G connects-toBdnf connects-toXanthine Oxidase connects-toIl 12 connects-toHemoglobin connects-toProstaglandins connects-toIron connects-toIl 4 connects-toIl 13 connects-toMagnesium connects-toZinc connects-toTgf Beta connects-toBaff connects-toIl 2 connects-toNmo connects-toLeptin connects-toAdiponectin connects-toResistin connects-toNatural Killer Cell connects-toIl 5 connects-toIl 23 connects-toIge connects-toT Helper Cell connects-toC5Ar1 connects-toPlasma Cell connects-toMast Cell connects-toFactor H connects-toC1 Esterase Inhibitor connects-toTransferrin connects-toTslp connects-toBradykinin connects-toErythropoietin connects-toHistamine connects-toPeriostin connects-toMelatonin connects-toTestosterone connects-toSerotonin connects-toProlactin connects-toOxytocin connects-toVasopressin connects-toSelenium connects-toIodine connects-toSodium connects-toPotassium connects-toCopper connects-toChloride connects-toSulfur connects-toNitrogen connects-toCarbon connects-toHydrogen connects-toOxygen connects-toPd 1 connects-toGlp 1 connects-toAngiotensin Ii connects-toWnt Beta Catenin connects-toRankl connects-toFibronectin connects-toNotch connects-toIgf 1 connects-toActivin A connects-toCgrp connects-toCalcitonin connects-toSubstance P connects-toInsulin Receptor connects-toAldosterone connects-toAndrogen Receptor connects-toNorepinephrine connects-toAdrenomedullin connects-toOsteopontin

Sources