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

Sources