| Autor: |
Gervais, Adrian, Le Floc'h, Corentin, Tom Le Voyer, Bizien, Lucy, Bohlen, Jonathan, Celmeli, Fatih, Al Qureshah, Fahd, Masson, Cécile, Rosain, Jérémie, Chbihi, Marwa, Lévy, Romain, Castagnoli, Riccardo, Rothenbuhler, Anya, Jouanguy, Emmanuelle, Qian Zhang, Shen-Ying Zhang, Béziat, Vivien, Bustamante, Jacinta, Puel, Anne, Bastard, Paul |
| Předmět: |
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| Zdroj: |
Proceedings of the National Academy of Sciences of the United States of America; 10/1/2024, Vol. 121 Issue 40, p1-12, 16p |
| Abstrakt: |
Human inborn errors of the type I IFN response pathway and auto-Abs neutralizing IFN-α, -β, and/or -ω can underlie severe viral illnesses. We report a simple assay for the detection of both types of condition. We stimulate whole blood from healthy individuals and patients with either inborn errors of type I IFN immunity or auto-Abs against type I IFNs with glycosylated human IFN-α2, -β, or -ω. As controls, we add a monoclonal antibody (mAb) blocking the type I IFN receptors and stimulated blood with IFN-γ (type II IFN). Of the molecules we test, IP-10 (encoded by the interferon-stimulated gene (ISG) CXCL10) is the molecule most strongly induced by type I and type II IFNs in the whole blood of healthy donors in an ELISA-like assay. In patients with inherited IFNAR1, IFNAR2, TYK2, or IRF9 deficiency, IP-10 is induced only by IFN-γ, whereas, in those with auto-Abs neutralizing specific type I IFNs, IP-10 is also induced by the type I IFNs not neutralized by the auto-Abs. The measurement of type I and type II IFN-dependent IP-10 induction therefore constitutes a simple procedure for detecting rare inborn errors of the type I IFN response pathway and more common auto-Abs neutralizing type I IFNs. [ABSTRACT FROM AUTHOR] |
| Databáze: |
Complementary Index |
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