![Figure 4. NF-κB activity in response to TNF-α and IL-1β. (A) IκBα degradation, detected by Western blotting and induced by TNF-α and IL-1β, for a healthy control subject (C+), an XD-IP patient, a hypomorphic NEMO EDA-ID-OL patient (X420W), the proband (P [D311G]), and an XR-MSMD NEMO patient (R319Q). Antibodies against GAPDH were used for normalization of protein levels on the Western blot. (B) NF-κB translocation was observed by EMSA with a labeled probe after exposure to TNF-α and IL-1β for various periods of time (0, 20, 40 minutes) for a healthy control subject (C+), an XD-IP patient, a hypomorphic NEMO EDA-ID-OL patient (X420W), the proband (P [D311G]), and an NEMO XR-MSMD patient (R319Q). (C) IL-10 production after TNF-α activation in whole blood from healthy control subjects, the proband (P [D311G]), and an NEMO XR-MSMD patient (R319Q). (D) IL-6 and IL-8 production, determined by ELISA after 24 hours of activation with TNF-α and IL-1β, for SV40-transformed fibroblast cell lines from a healthy control subject (C+), an XD-IP patient, an NEMO EDA-ID-OL patient (X420W), the proband (P [D311G]), and an NEMO XR-MSMD patient (R319Q). These results are representative of 2 independent experiments.](https://ash.silverchair-cdn.com/ash/content_public/journal/blood/118/4/10.1182_blood-2010-10-315234/4/zh89991174700004.jpeg?Expires=1724242973&Signature=1YWufup-34JGekCvz9ZmXnWqrLk~se~3FkJdIyme7FlcXxJwOqosamKdsAc7Z6~J4dPzTAYDU~MfD8onL1oNvtVKK04A2t8hnt0SRT5UFSEEwKzY0rNRrV1dhf8QvThCc1WeQVUbfxI7ho76F4IAb0nU08dKBZgNaNsndDgrb1hY6ylGAqTRXYXff6dTgSHirndVM2LKg7jmh~YqqoMN58jjVLb072~7w~xQiqJoPZoFlb9JtghYET3RSjs7QjNpyb-yAz9UZXm3g7o-KmOn1RR~y54yjsHutrhBitZGTF-0iSTvBXU1GXA8YVR8AXDeCRmnxdqtf1ch6YazcbmoPA__&Key-Pair-Id=APKAIE5G5CRDK6RD3PGA)
NF-κB activity in response to TNF-α and IL-1β. (A) IκBα degradation, detected by Western blotting and induced by TNF-α and IL-1β, for a healthy control subject (C+), an XD-IP patient, a hypomorphic NEMO EDA-ID-OL patient (X420W), the proband (P [D311G]), and an XR-MSMD NEMO patient (R319Q). Antibodies against GAPDH were used for normalization of protein levels on the Western blot. (B) NF-κB translocation was observed by EMSA with a labeled probe after exposure to TNF-α and IL-1β for various periods of time (0, 20, 40 minutes) for a healthy control subject (C+), an XD-IP patient, a hypomorphic NEMO EDA-ID-OL patient (X420W), the proband (P [D311G]), and an NEMO XR-MSMD patient (R319Q). (C) IL-10 production after TNF-α activation in whole blood from healthy control subjects, the proband (P [D311G]), and an NEMO XR-MSMD patient (R319Q). (D) IL-6 and IL-8 production, determined by ELISA after 24 hours of activation with TNF-α and IL-1β, for SV40-transformed fibroblast cell lines from a healthy control subject (C+), an XD-IP patient, an NEMO EDA-ID-OL patient (X420W), the proband (P [D311G]), and an NEMO XR-MSMD patient (R319Q). These results are representative of 2 independent experiments.
