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. 2023 Nov 8;623(7988):803–813. doi: 10.1038/s41586-023-06717-x

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© The Author(s) 2023

Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/.

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Extended Data Fig. 7 — (a-b) Luciferase-based neutralization assay for detecting auto-Abs neutralizing 10 ng/mL IFN-α2 (a) or IFN-ω (b) in patients with the three inborn errors of NF-κB2, APS-1 and PAD patients, positive controls (C+), and healthy controls (HC). (c) Correlation between the detection of auto-Abs against IFN-α2 by Gyros (x-axis) and results for the luciferase-based neutralization assay (y-axis) after stimulation with 10 ng/mL IFN-α2. The dotted line represents the cutoffs for detection (A.U. value > 50) or neutralization (induction <5). (d-e) Proportion of patients with auto-Abs neutralizing type I IFNs at 10 ng/mL or 100 pg/mL among patients with a p52^LOF/IκBδ^GOF variant (d), and APS-1 patients (e). (f) Proportion of patients with AAN-I-IFNs among patients carrying a missense or pLOF p52^LOF/IκBδ^GOF variant. (g) Age distribution of patients with the three inborn errors of NF-κB2, AR RelB or NIK deficiency, or APS-1 according to the presence or absence of AAN-I-IFNs in plasma. (h) Detection of IgG auto-Abs against IFN-α2 by Gyros in patients with inborn errors of NIK, RelB, BAFF and CD40L. (i-j) Luciferase-based neutralization assay for detecting auto-Abs neutralizing 10 ng/mL IFN-α2 (i) or IFN-ω (j) in patients with inborn errors of NIK, RelB, BAFF and CD40L. (k) Proportion of patients with auto-Abs neutralizing type I IFNs at 10 ng/mL or 100 pg/mL in patients with AR RelB deficiency. (l-m) Luciferase-based neutralization assay for detecting auto-Abs neutralizing 100 pg/mL IFN-α2 (l) or IFN-ω (m) in patients with inborn errors of the canonical NF-κB pathway. DN = dominant-negative. (n) Detection of auto-Abs neutralizing 100 pg/mL IFN-α2 or IFN-ω in patients with inborn errors of the alternative NF-κB pathway post-HSCT (n = 7) versus children with inborn errors of T-cell intrinsic immunity [(SCID, n = 3, CID, n = 1), neutrophil-intrinsic immunity (chronic granulomatous disease, CGD, n = 10), cytotoxicity (familial hemophagocytic lymphohistiocytosis, HLH, n = 3), erythrocyte function (β-thalassaemia, n = 3)] who underwent HSCT (Hematop. IE, n = 20) (left panel), with the time interval between HSCT and plasma collection (right panel).