Type I IFN-related NETosis in ataxia telangiectasia and Artemis deficiency


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Gul E., Sayar E. H. , Gungor B., Eroglu F. K. , Surucu N., Keleş S., ...More

JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY, vol.142, pp.246-257, 2018 (Peer-Reviewed Journal) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 142
  • Publication Date: 2018
  • Doi Number: 10.1016/j.jaci.2017.10.030
  • Journal Name: JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY
  • Journal Indexes: Science Citation Index Expanded, Scopus
  • Page Numbers: pp.246-257
  • Keywords: Primary immunodeficiencies, autoinflammation, ataxia telangiectasia, Artemis deficiency, type I IFN, interferonopathy, neutrophil extracellular traps, NETosis, NEUTROPHIL EXTRACELLULAR TRAPS, SYSTEMIC-LUPUS-ERYTHEMATOSUS, ACUTE MYOCARDIAL-INFARCTION, COMBINED IMMUNODEFICIENCY, CUTANEOUS GRANULOMAS, MITOCHONDRIAL-DNA, DCLRE1C MUTATIONS, DISEASE, DAMAGE, INTERFERONOPATHIES

Abstract

Background: Pathological inflammatory syndromes of unknown etiology are commonly observed in ataxia telangiectasia (AT) and Artemis deficiency. Similar inflammatory manifestations also exist in patients with STING-associated vasculopathy in infancy (SAVI).