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Impaired thymic AIRE expression underlies autoantibodies against type I IFNs in humans with inborn errors of the alternative NF-κB pathway

Patients with autoimmune polyendocrinopathy syndrome type 1 (APS-1) caused by autosomal recessive AIRE deficiency display autoantibodies (auto-Abs) neutralizing type I IFNs, conferring a predisposition to life-threatening COVID-19 pneumonia. We report that patients with autosomal recessive NIK or Re...

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Detalles Bibliográficos
Autores principales: Voyer, Tom Le, Gervais, Adrian, Rosain, Jérémie, Parent, Audrey V., Cederholm, Axel, Roifman, Chaim, Landegren, Nils, Anderson, Mark S., Casanova, Jean-Laurent, Puel, Anne
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier Inc. 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10203942/
http://dx.doi.org/10.1016/j.clim.2023.109369