Scopus Eşleşmesi Bulundu
89
Atıf
379
Cilt
🔓
Açık Erişim
Scopus Yazarları: Darawan Rinchai, Zhiyong Liu, Iolanda Jordan, Gulsum Iclal Bayhan, Camille Beaufils, Jie Chen, Tomi Lazarov, Lazaro Lorenzo, Jordi Anton, Flore Rozenberg, Xavier Duval, Ahmad Yatim, Beihua Dong, Yann Aquino, Estelle Talouarn, Peng Zhang, Weite Lei, Milena Hasan, H. Heinrich Hoffmann, Eleftherios Michailidis, Kaya Bilguvar, Carlos Flores, Marcela Moncada-Velez, Francesca Pala, Yi Hao Chan, Serkan Belkaya, Selket Delafontaine, Danyel Lee, Jérémie Le Pen, Masato Ogishi, Rémi Pescarmona, Marion Moreews, Yoann Seeleuthner, Shrikant Mane, Yu Zhang, Rasheed Bailey, Baptiste Milisavljevic, Benedetta Bigio, Adrian Gervais, Romain Lévy, Anna Lena Neehus, Marie Paule Morin, Rosa Maria Pino-Ramirez, Magali Perret, Sefika Elmas Bozdemir, Lucy Bizien, Aurelie Bisiaux, Christina Gaughan, Abhishek Asthana, Valentina Libri, Joseph M. Luna, Fabrice Jaffré, Andrés Augusto Arias, Agatha Schlüter, Tom Le Voyer, Marie Materna, Jérémie Rosain, Jessica Peel, Julie Toubiana, Fanny Bajolle, Victoria Fumadó, Marta L. DeDiego, Nadhira Fidouh, Jordi Perez-Tur, Shuibing Chen, Todd Evans, Frédéric Geissmann, Pierre Lebon, Susan R. Weiss, Bertrand Boisson, Qian Zhang, Luigi Notarangelo, Rebeca Pérez de Diego, Richard P. Lifton, Aurélie Cobat, Laia Alsina, Isabelle Meyts, Aurora Pujol, Vanessa Sancho-Shimizu, Paul Bastard, Vivien Béziat, Carlos Rodriguez-Gallego, Helen C. Su, Elie Haddad, Robert H. Silverman, Damien Bonnet, Qiang Pan-Hammarström, Anna M. Planas, Anne Puel, Emmanuelle Jouanguy, Sevgi Keles, Charles M. Rice, Filomeen Haerynck, Clifton L. Dalgard, Jacinta Bustamante, Stéphanie Boisson-Dupuis, Tom Maniatis, Laurent Abel, Alexandre Belot, Lluis Quintana-Murci
Özet
Multisystem inflammatory syndrome in children (MIS-C) is a rare and severe condition that follows benign COVID-19. We report autosomal recessive deficiencies of OAS1, OAS2, or RNASEL in five unrelated children with MIS-C. The cytosolic double-stranded RNA (dsRNA)-sensing OAS1 and OAS2 generate 2'-5'-linked oligoadenylates (2-5A) that activate the single-stranded RNA-degrading ribonuclease L (RNase L). Monocytic cell lines and primary myeloid cells with OAS1, OAS2, or RNase L deficiencies produce excessive amounts of inflammatory cytokines upon dsRNA or severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) stimulation. Exogenous 2-5A suppresses cytokine production in OAS1-deficient but not RNase L-deficient cells. Cytokine production in RNase L-deficient cells is impaired by MDA5 or RIG-I deficiency and abolished by mitochondrial antiviral-signaling protein (MAVS) deficiency. Recessive OAS-RNase L deficiencies in these patients unleash the production of SARS-CoV-2-triggered, MAVS-mediated inflammatory cytokines by mononuclear phagocytes, thereby underlying MIS-C.
Makale Bilgileri
Dergi
Science
ISSN
0036-8075
Yıl
2023
/ 2. ay
Cilt / Sayı
379
/ 6632
Sayfalar
3627 – 3627
Makale Türü
Özgün Makale
Hakemlik
Hakemli
Endeks
SCI-Expanded
JCR Quartile
Q1
Yayın Dili
İngilizce
Kapsam
Uluslararası
Toplam Yazar
352 kişi
Erişim Türü
Basılı+Elektronik
Erişim Linki
Makaleye Git
Alan
Sağlık Bilimleri Temel Alanı
Çocuk Enfeksiyon Hastalıkları
YÖKSİS Yazar Kaydı
Yazar Adı
ELMAS BOZDEMİR ŞEFİKA, BAYHAN GÜLSÜM İCLAL, BELKAYA SERKAN, KELEŞ SEVGİ, METİN AKCAN ÖZGE, AKÇAY NİHAL, ALKAN GÜLSÜM, YOSUNKAYA ALPER, GAYRETLİ AYDIN ZEYNEP GÖKÇE, Aytekin Caner, EROL AYTEKİN SELMA, ORBAK ZERRİN, AYDEMİR ŞEHNAZ, AYTEKİN GÖKHAN, ÇELİK JALE BENGİ, COŞKUNER TANER, BURSAL DURAMAZ BURCU, EMİROĞLU MELİKE, ERDENİZ EMİNE HAFİZE, GÜL YAHYA, GÜLHAN BELGİN, KILIÇ GÜLTEKİN SARA ŞEBNEM, HATİPOĞLU NEVİN, KANDEMİR BAHAR, KANIK YÜKSEK SALİHA, KAPAKLI HASAN, KARBUZ ADEM, KASAPÇOPUR ÖZGÜR, KENDİR DEMİRKOL YASEMİN, KILIÇ ÖMER, KILIÇ AHMET OSMAN, TÜTER ÖZ ŞADİYE KÜBRA, ÖZÇELİK HASAN TAYFUN, AKYÜZ ÖZKAN ESRA, ÖZKAYA PARLAKAY ASLINUR, POLAT MELTEM, ŞEVKETOĞLU ESRA, TANIR GÖNÜL, HANÇERLİ TÖRÜN SELDA, UYAR EMEL, VATANSEV HÜLYA, YAHŞİ AYSUN, YARAR VOLKAN, YEŞİLBAŞ OSMAN, YILDIZ MEHMET
YÖKSİS ID
7070872
Hızlı Erişim
Metrikler
Scopus Atıf
89
JCR Quartile
Q1
Yazar Sayısı
352