Scopus
🔓 Açık Erişim YÖKSİS DOI Eşleşti
SJR Q1
Autoantibodies against type I IFNs in humans with alternative NF-κB pathway deficiency
Nature · Kasım 2023
Özet
Patients with autoimmune polyendocrinopathy syndrome type 1 (APS-1) caused by autosomal recessive AIRE deficiency produce autoantibodies that neutralize type I interferons (IFNs)1,2, conferring a predisposition to life-threatening COVID-19 pneumonia3. Here we report that patients with autosomal recessive NIK or RELB deficiency, or a specific type of autosomal-dominant NF-κB2 deficiency, also have neutralizing autoantibodies against type I IFNs and are at higher risk of getting life-threatening COVID-19 pneumonia. In patients with autosomal-dominant NF-κB2 deficiency, these autoantibodies are found only in individuals who are heterozygous for variants associated with both transcription (p52 activity) loss of function (LOF) due to impaired p100 processing to generate p52, and regulatory (IκBδ activity) gain of function (GOF) due to the accumulation of unprocessed p100, therefore increasing the inhibitory activity of IκBδ (hereafter, p52LOF/IκBδGOF). By contrast, neutralizing autoantibodies against type I IFNs are not found in individuals who are heterozygous for NFKB2 variants causing haploinsufficiency of p100 and p52 (hereafter, p52LOF/IκBδLOF) or gain-of-function of p52 (hereafter, p52GOF/IκBδLOF). In contrast to patients with APS-1, patients with disorders of NIK, RELB or NF-κB2 have very few tissue-specific autoantibodies. However, their thymuses have an abnormal structure, with few AIRE-expressing medullary thymic epithelial cells. Human inborn errors of the alternative NF-κB pathway impair the development of AIRE-expressing medullary thymic epithelial cells, thereby underlying the production of autoantibodies against type I IFNs and predisposition to viral diseases.
YÖKSİS Kayıtları
Autoantibodies against type I IFNs in humans with alternative NF-κB pathway deficiency
Nature · 2023 SCI-Expanded
Prof. Dr. MELİKE EMİROĞLU →
Autoantibodies against type I IFNs in humans with alternative NF-κB pathway deficiency
Nature · 2023 SCI-Expanded
Prof. Dr. JALE BENGİ ÇELİK →
YÖKSİS Kayıtları — ISSN Eşleşmesi
Bu dergide (ISSN eşleşmesi) kurumun 5 kaydı bulundu.
YÖKSİS Kayıtları — ISSN Eşleşmesi
Bu dergide (ISSN eşleşmesi) kurumun 5 kaydı bulundu.
Human genetic and immunological determinants of critical COVID-19 pneumonia
2022 ISSN: 0028-0836 SCI-Expanded Q1
Doç. Dr. GÜLSÜM ALKAN →
Autoantibodies against type I IFNs in humans with alternative NF-κB pathway deficiency
2023 ISSN: 0028-0836 SCI-Expanded Q1
Prof. Dr. JALE BENGİ ÇELİK →
Human TMEFF1 is a restriction factor for herpes simplex virus in the brain
2024 ISSN: 0028-0836 SCI-Expanded Q1
Prof. Dr. MELİKE EMİROĞLU →
Exome sequencing reveals low-frequency and rare variant contributions to multiple sclerosis susceptibility in Turkish families
2025 ISSN: 0028-0836 SCI-Expanded Q1
Prof. Dr. HALUK GÜMÜŞ →
COVID-19-associated neurological and psychological manifestations
2025 ISSN: 0028-0836 SCI-Expanded
Prof. Dr. ŞEREFNUR ÖZTÜRK →
Makale Bilgileri
Dergi
Nature
Toplam Atıf
95 atıf
· Scopus
ISSN00280836
Yayın TarihiKasım 2023
Cilt / Sayfa623 · 803-813
Scopus ID2-s2.0-85176091832
Erişim🔓 Açık Erişim
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Aarhus Universitet
Aarhus Denmark
Academy of Athens
Athens Greece
Akademiska Sjukhuset
Uppsala Sweden
A-Star, Infectious Disease Lab
Singapore City Singapore
Bilkent Üniversitesi
Ankara Turkey
Centre Hospitalier Universitaire Ibn Rochd
Casablanca Morocco
Centre Hospitalier Universitaire Vaudois
Lausanne Switzerland
Centro de Investigación Biomédica en Red de Enfermedades Raras
Madrid Spain
Chang Gung University
Taoyuan Taiwan
Collège de France
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College of Medicine
Riyadh Saudi Arabia
Columbia University Irving Medical Center
New York United States
Consejo Superior de Investigaciones Científicas
Madrid Spain
Dasman Diabetes Institute
Dasman Kuwait
Departement Microbiologie, Immunologie en Transplantatie
Leuven Belgium
Department of Paediatrics
Melbourne Australia
École Polytechnique Fédérale de Lausanne
Lausanne Switzerland
Faculdades Pequeno Príncipe
Curitiba Brazil
Facultad de Medicina Clinica Alemana Universidad del Desarrollo
Las Condes Chile
Fondazione IRCCS Policlinico San Matteo
Pavia Italy
Fudan University
Shanghai China
Garvan Institute of Medical Research
Sydney Australia
Hackensack Meridian Health
Edison United States
Hiroshima University Graduate School of Biomedical and Health Sciences
Hiroshima Japan
Hopital Cochin AP-HP
Paris France
Hôpital Necker Enfants Malades
Paris France
Hôpital Saint-Antoine
Paris France
Hôpital Saint-Louis
Paris France
Hôpital Universitaire Pitié Salpêtrière
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Santiago Chile
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New York United States
Inserm
Paris France
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Barcelona Spain
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Hospitalet de Llobregat Spain
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Seattle United States
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Naples Italy
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Tokyo Japan
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Madrid Spain
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Granadilla de Abona Spain
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Rome Italy
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Stockholm Sweden
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Munich Germany
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Leuven Belgium
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Singapore City Singapore
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Brno Czech Republic
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Samsun Turkey
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Poznan Poland
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New York United States
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Milan Italy
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Konya Turkey
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Kyiv Ukraine
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Paris France
Tartu Ülikool
Tartu Estonia
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Tehran Iran
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Tel Aviv-Yafo Israel
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Hong Kong Hong Kong
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San Francisco United States
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Medellin Colombia
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Sao Paulo Brazil
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Santa Maria de Guia de Gran Canaria Spain
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Brescia Italy
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Rome Italy
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Ghent Belgium
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Freiburg im Breisgau Germany
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Varna Bulgaria
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Edmonton Canada
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Sharjah United Arab Emirates
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Kensington Australia
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Uppsala Sweden
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Barcelona Spain
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Penrith Australia
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Scimago Dergi (ISSN Eşleşmesi)
Nature
Q1
SJR Skoru19,713
H-Index1495
YayıncıNature Research
ÜlkeUnited Kingdom
Multidisciplinary (Q1)
Metrikler
95
Atıf