Type I IFN-related NETosis in ataxia telangiectasia and Artemis deficiency
JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY, vol.142, no.1, pp.246-257, 2018 (SCI-Expanded, Scopus)
- Publication Type: Article / Article
- Volume: 142 Issue: 1
- 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 (SCI-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
- Open Archive Collection: AVESIS Open Access Collection
- Hacettepe University Affiliated: Yes
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).