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Article

Aicardi-Goutières syndrome: A monogenic type I interferonopathy.

Scandinavian journal of immunology - 1 Oct 2023

Liu Anran, Ying Songcheng

Abstract excerpt

Aicardi-Goutières syndrome (AGS) is a rare monogenic autoimmune disease that primarily affects the brains of children patients. Its main clinical features include encephalatrophy, basal ganglia calcification, leukoencephalopathy, lymphocytosis and increased interferon-α (IFN-α) levels in the patient's cerebrospinal fluid (CSF) and serum. AGS may be caused by mutations in any one of nine genes (TREX1, RNASEH2A,...

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Identifiers

PMID: 37515439
DOI: 10.1111/sji.13314

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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