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

Gul, Ersin; Sayar, Esra; Gungor, Bilgi; Eroglu, Fehime; Surucu, Naz; Keleş, Sevgi; Güner, Şükrü; Findik, Siddika; Alpdundar, Esin; Ayanoğlu, İHSAN; Kayaoğlu, BAŞAK; Geckin, Busra; Sanli, Hatice; Kahraman, Tamer; Yakicier, Cengiz; Müftüoğlu, Meltem; Oğuz, Berna; Çağdaş Ayvaz, Deniz; Gürsel, İhsan; Özen, Seza; Reisli, İsmail; Gürsel, MAYDA

doi:10.1016/j.jaci.2017.10.030

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

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)

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).