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STING signals to NF-κB from late endolysosomal compartments using IRF3 as an adaptor

Abstract

NF-κB is central for activation of immune responses. Cytosolic DNA activates the cGAS–STING pathway to induce type I interferons (IFNs) and signaling through NF-κB, thus instigating host defenses and pathological inflammation. However, the mechanism underlying STING-induced NF-κB activation is unknown. Here we report that STING activates NF-κB in a delayed manner, following exit from the Golgi to endolysosomal compartments. Activation of NF-κB is dependent on the IFN-inducing transcription factor IRF3 but is independent of type I IFN signaling. This activation pattern is evolutionarily conserved in tetrapods. Mechanistically, the monomer IRF3 is recruited to STING pS358, with delayed kinetics relative to IRF3 recruitment to STING pS366, which promotes type I IFN responses. IRF3 engagement with STING pS358 induces trafficking to late endolysosomal compartments, supporting recruitment of TRAF6 and activation of NF-κB. We identify a TRAF6 binding motif in IRF3 that facilitates recruitment of TRAF6. This work defines a signaling surface on STING and a function for IRF3 as an adaptor in immune signaling. These findings indicate that STING signaling to NF-κB is enabled only within a short time window between exit from the Golgi and lysosomal degradation, possibly limiting inflammation under homeostatic and danger-sensing conditions.

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Fig. 1: STING-driven NF-κB activation occurs at endolysosomes.
Fig. 2: IRF3 is essential for the STING-triggered inflammatory response in vitro.
Fig. 3: IRF3 is essential for the STING-triggered inflammatory response in vivo.
Fig. 4: IRF3 functions as an essential adaptor recruiting TRAF6 to STING.
Fig. 5: Conservation of IRF3-mediated STING-driven NF-κB activation across tetrapods.
Fig. 6: STING S358 is essential for inflammatory gene expression in vitro and in vivo.
Fig. 7: IRF3 docks at STING pS358 to enable TRAF6 recruitment.
Fig. 8: IRF3–STING S358 mediate STING post-Golgi trafficking distinct from STING S366.

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Data availability

The sequencing data reported in this article are deposited in the Gene Expression Omnibus database under accession code GSE305060. All other data are available in the supplementary and article files. Source data are provided with this paper.

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Acknowledgements

The laboratory of S.R.P. is supported by Independent Research Fund Denmark (0134-00008B and 1026-00003B), the Lundbeck Foundation (R359-2020-2287), the Novo Nordisk Foundation (NNF18OC0030274, NNF20OC0064301 and NNF20OC0063436) and the European Research Council (786602). T.H.M. is supported by the Independent Research Fund Denmark (0134-00006B) and by the Novo Nordisk Foundation (NNF20OC0064890 and NNF21OC0067157). Research in the laboratory of Pingwei Li was supported by NIH grant AI145287 and the Welch Foundation grant A-2107. ImageStream and flow cytometry were performed at the FACS Core Facility, Aarhus University, Denmark, and confocal imaging was performed at the Bioimaging Core Facility, Aarhus University, Denmark. Finally, we wish to thank the individuals who participated in the study and provided blood samples or skin biopsies for generation of fibroblasts.

Author information

Authors and Affiliations

Authors

Contributions

A.P. first observed the essential role for IRF3 in STING-induced NF-κB activation. B.-c.Z. and S.R.P. conceived the idea and designed the experiments. B.-c.Z. determined the mechanism. B.-c.Z., A.P., L.S.R., Y.L., R.N., M.I., L. Hu, M.K.S., S.L., M.M., Y.C., J.Z., K.M., Z.G., E.A.T., J.H.M., R.V., M.B.I., S.A. and R.Z. performed the experiments. L. Henneman generated transgenic mice. X.D. and J.-r.H. performed the bioinformatics analysis. M.-L.F., M.K.T., M.R.J., C.O., T.S.D., A.F. and T.W. provided materials and specific input to the paper. T.H.M. and M.-L.F. were responsible for ethics permission and for obtaining patient cells. C.B.F.A., D.D.N., F.R., J.P.-Y.T., M.K.T., P.M., J.G.M., R.O.B., T.H.M., P.L. and S.R.P. supervised the experiments. B.-c.Z., A.P. and S.R.P. wrote the paper.

Corresponding authors

Correspondence to Bao-cun Zhang or Søren R. Paludan.

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The authors declare no competing interests.

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Nature Immunology thanks Stefan Bauer and the other, anonymous, reviewer(s) for their contribution to the peer review of this work. Primary Handling Editor: Nick Bernard, in collaboration with the Nature Immunology team. Peer reviewer reports are available.

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Extended data

Extended Data Fig. 1 Differential kinetics of STING-mediated activation of IRF3 and NF-κB p65 pathways.

a, b, ImageStream Analysis. HaCaT cells were treated with 4ug/ml dsDNA a, or HSV-2 b, for various time points, and then probed for p65 and IRF3 using Alexa Fluor® 647 conjugated Mouse Anti-Human IRF-3 and Phycoerythrin PE,-conjugated rabbit anti-NF-кB p65 antibodies. Nuclear translocation of p65 and IRF3 was analyzed by ImageStream (n = 3). c, d, Fibroblast cells c, or THP1 cells d, were treated with cGAMP for 0, 1, and 4 h, and then probed for p65 and IRF3 using Alexa Fluor® 647 conjugated Mouse Anti-Human IRF-3 and Phycoerythrin PE,-conjugated rabbit anti-NF-кB p65 antibodies. Nuclear translocation of p65 and IRF3 was analyzed by ImageStream(n = 3). e, NF-кB/ISRE Driven Reporter Assay. HEK293T cells expressing STING were co-transfected with 50 ng of NF-кB/ISRE promoter luciferase reporter and 30 ng of β-actin Renilla reporter. After 24 h of transfection, cells were stimulated with cGAMP for indicated time points(n = 3). f, g, HaCaT cells were treated with poly I:C with different concentrations f, or at different time points g, and then probed for p65 and IRF3 using Alexa Fluor® 647 conjugated Mouse Anti-Human IRF-3 and Phycoerythrin PE,-conjugated rabbit anti-NF-кB p65 antibodies. Nuclear translocation of p65 and IRF3 was analyzed by ImageStream (n = 3). h, Upper panel, Illustration of ImageStream analysis strategy to study if the IRF3 and NF-κB p65 pathways are activated in the same or different cells. Bottom panel, ImageStream analysis of IRF3 and p65 nuclear translocation levels in THP1 cells treated with cGAMP (100ug/ml), dsDNA (5 μg/ml) or HSV-2 (MOI = 3) (n = 3). i, NF-κB/ISRE driven reporter assay. HEK293T cells were co-transfected with 100 ng of STING-WT/S366A/ΔCTT and 50 ng of the NF-κB/ISRE promoter luciferase reporter and 30 ng of the β-actin Renilla reporter (n = 3). j, HaCaT cells were pre-treated with 2ug/ml Brefeldin A (BFA) for 1 h and then stimulated with cGAMP for 0, 1, and 4 h. The levels of the indicated proteins were determined by immunoblotting. k, Immunoblot analysis of HaCaT cells treated with vehicle or cGAMP following electroporation with Cas9 protein and each of the indicated sgRNAs. Results are presented as mean ± SD (ai). P values were calculated using a two-sided, one-way ANOVA with Dunnett’s multiple comparisons test (ad, and g), and two-way ANOVA with Sidak’s multiple comparisons test (e) and Tukey’s multiple comparisons test (e). All results presented in this figure are representative from 3 independent experiments with similar results.

Source data

Extended Data Fig. 2 IRF3 specifically mediates the STING-induced NF-κB activation.

a, WT and p65-deficient HaCaT cells were treated with cGAMP for 0 and 4 h. ImageStream analyzed the nuclear translocation of the IRF3 (n = 3). b, WT, IRF3- or p65-ko HaCaT cells treated were stimulated with cGAMP. The mRNA level of the indicated gene was determined by qRT-PCR (n = 3). c, d, HaCaT cells treated with Cas9 and AAVS1, IRF3 or STING gRNAs were stimulated with 4ug/ml dsDNA c, or 4 Gy X-Ray d, The level of the indicated proteins was determined by immunoblotting. e, f, NF-κB/ISRE driven reporter assay. HEK293T cells were co- transfected with 1 ng, 10 ng, or 100 ng of MAVS e or TRIF f encoding plasmids and 50 ng of the NF-κB/ISRE promoter luciferase reporter and 30 ng of the β-actin Renilla reporter (n = 3). g, h, ImageStream analysis nuclear location of NF-κB p65 in Control and IRF3-depleted HaCaT cells after stimulation with poly(I:C) (500 ng/ml) or TNFα (20 ng/ml) or for 1 and 4 h (n = 3). Results are presented as mean ± SD (a, b and eh). P values were calculated using a two-sided, two-way ANOVA with Sidak’s multiple comparisons test (a, and eh) and T Dunnett’s multiple comparisons test (b). All results presented in this figure are representative from 3 independent experiments with similar results.

Source data

Extended Data Fig. 3 IRF3 is important for STING-induced NF-κB activation.

a, Primary human fibroblasts treated with Cas9 and AAVS1 (control) or IRF3 gRNAs were stimulated with vehicle or cGAMP for 4 h. The levels of the indicated proteins were determined by immunoblotting. b, Primary MEFs from WT and Irf3−/− mice were stimulated with vehicle or cGAMP. The mRNA levels of Il6 and Cxcl10 were determined by qRT-PCR (n = 3). c, WT and IRF3-, IFNAR2-deficient HaCaT cells were treated with cGAMP for different time points. The levels of the indicated proteins were determined by immunoblotting. d, WT and IRF3-, IFNAR2-deficient HaCaT cells were treated with vehicle or cGAMP for 4 h. Nuclear translocation of p65 was analyzed by ImageStream (n = 3). e, HaCaT cells deficient in IRF3 were rescued with IRF3-WT/K193R, and then the cells were stimulated with vehicle or cGAMP for 4 h. The levels of the indicated proteins were determined by immunoblotting. f, HaCaT cells treated with Cas9 and AAVS1 or Bax gRNAs were stimulated with vehicle or cGAMP for 4 h. The levels of the indicated proteins were determined by immunoblotting. Results are presented as mean ± SD (b, d). All results presented in this figure are representative from 3 independent experiments with similar results.

Source data

Extended Data Fig. 4 Role for IRF3 in STING-dependent inflammatory response in human and mice.

a, Representative images of C57BL/6-Wt, Irf3−/−, Ifnr1−/−, and Stinggt/gt mice after ionizing irradiation for 3 months. b, Levels of mRNA of Tnfa, Il6, and Cxcl10 in spleens from Wt, Irf3−/−, and Irf3R278Q/R278Q mice treated with LPS (5 mg/kg body weight) for 6 h. Mock-Wt and Mock- Irf3−/− (n = 4 mice), Mock- Irf3R278Q/R278Q (n = 6 mice), LPS-Wt (n = 5 mice), LPS- Irf3−/− and LPS- Irf3R278Q/R278Q (n = 6 mice). Results are presented as mean ± SD. P values were calculated using a two-sided, Brown-Forsythe ANOVA test followed by Dunnett’s T3 multiple comparisons test. c, Box plots of The Cancer Genome Atlas TCGA, RNA expression profiles in KIRC, CESC and LUSC. The highest and lowest 25% of STING expression were analyzed by comparing STING-high and STING-low groups. Statistical analysis was performed using a two-tailed Mann-Whitney test. The upper and lower ends of the boxes represent the upper and lower quartiles, and the horizontal line inside the box is the median of the dataset. The whiskers indicate the upper and lower extremes of the dataset.

Source data

Extended Data Fig. 5 IRF3 Ser396 and the TRAF6 binding motif synergize to mediates STING-induced NF-κB activation.

a, HaCaT cells deficient in IRF3 were rescued with IRF3 WT, R211Q, R285Q, S386A, or S396A. and stimulated with vehicle or cGAMP. The levels of the indicated proteins were determined by immunoblotting. b, Murine embryonic fibroblasts (MEFs) were treated with control (scrambled gRNA)- or TRAF6-targeting gRNAs. The cells were treated with cGAMP for 6 h and total RNA was isolated for RT-qPCR analysis. Data show normalized levels of Tnfa and Il6 mRNA measured by RT-qPCR (n = 3). c, THP1 cells were treated with control (scrambled gRNA)- and TRAF6-targeting gRNAs and treated with cGAMP for 2 h. Lysates were probed for the indicated proteins measured by immunoblotting. d, SDS-PAGE for verification of protein purification and chromatogram of size exclusion chromatography of TRAF6-MATH domain or IRF3 peptide. e, Schematic of STING chimera CTT constructs with/without TRAF6 binding motif from human IRF3 or fish STING CTT. f, FLAG was immunoprecipitated from HEK293T cells with FLAG-STING WT/ + TRAF6 binding motif transfected with Myc-TRAF6. Precipitates were immunoblotted with anti-Myc. g, NF-κB/ISRE-driven reporter assay. HEK293T cells expressing STING-WT/STING + TRAF6 binding motif were co-transfected with 50 ng of the NF-κB/ISRE promoter luciferase reporter and 30 ng of the β-actin Renilla reporter (n = 3). h, WT and IRF3-deficient HEK293T cells were co-transfected with Flag-tagged TRAF6 and HA-tagged Ub-K63 only. After transfection for 24 h, the cells were stimulated with vehicle or cGAMP. Co-immunoprecipitation and immunoblot analysis were performed with the indicated antibodies. i, HEK293T cells with stable expression of STING and IRF3-WT or IRF3 mutants as indicated were transfected with Flag-tagged TRAF6. FLAG was immunoprecipitated from the cells, and the precipitates were immunoblotted with the indicated antibodies. Results are presented as mean ± SD (b and g). P values were calculated using a two-sided, two-way ANOVA with Tukey’s multiple comparisons test (b and g). All results presented in this figure are representative from 3 independent experiments with similar results.

Source data

Extended Data Fig. 6 NF-κB activation via TRAF6-IRF3-STING pathway is conserved in terrestrial animals.

a, FLAG was immunoprecipitated from HEK293T cells co-transfected with Myc-TRAF6 and different species of STING, and stimulated with vehicle or cGAMP for 6 h. The precipitates were immunoblotted with anti-Myc. b, Quantification of Myc-TRAF6 by western blotting. The band intensity of Myc-TRAF6 was plotted after normalization to the Flag-STING signal of the same lane. c, The activation of STING-NF-κB mediated by TRAF6-IRF3 is conserved in terrestrial animals. The mechanism of regulation involves IRF3 docking at STING, which acts as an adaptor to recruit TRAF6. This mechanism is highly conserved from amphibians to mammals. The affinity level between STING and IRF3 determines the preference of species STING to activate IFN or NF-κB. In lower species, STING has a higher affinity for IRF3, leading to the induction of a higher level of NF-κB response. In contrast, higher species STING conversely exhibit a lower propensity. d, Phylogeny analysis of type I interferon genes in terrestrial animals. e, HaCaT cells deficient in STING were reconstituted with human/mouse STING-WT or mutants as indicated. The cells were stimulated with cGAMP delivered by Digitonin and then incubated at 37 °C or 21 °C for 4 h. The levels of the indicated proteins were determined by immunoblotting. f, PBMCs from carp and mouse were stimulated with vehicle or cGAMP at 37 °C or 21 °C. Total RNA was isolated and analyzed by RT-qPCR. Results are presented as mean ± SD (n = 3). Results presented in a, b, e, f are representative from 3 independent experiments with similar results.

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Extended Data Fig. 7 IRF3 docking at STING pS358 facilitates NF-κB activation.

a, Alignment of the C-terminal tail CTT, sequences of STING from different species. b, Wild type THP1 and THP1 STING KO cells were treated with 50 µg/mL cGAMP for the indicated time intervals. Whole-cell lysates were prepared and analyzed by SDS–PAGE followed by immunoblotting with the antibodies shown. c, HaCaT cells treated with Cas9 and scramble gRNA (control), TBK1 gRNAs or IKKƐ gRNAs were stimulated with vehicle or cGAMP for 5 h. Levels of the indicated proteins were determined by immunoblotting. d, Illustration of the design for generation of mice carrying the Sting S357A amino acid substitution (top panel) and mice carrying both S357A and S365A substitutions (bottom panel) by CRISPR/Cas9 microinjection in C57BL/6 J zygotes as described in the Methods section. The bottom part of each panel shows Sanger sequencing chromatograms from genome-edited mice confirming the introduced codon changes. e, RT-qPCR analysis of Il6, and Cxcl10 in total RNA isolated from Wt, StingS357A/S357A, StingS365A/S365A and StingS357A,S365A/S357A,S365A BMMs after stimulation with vehicle, cGAMP (20ug/ml) or diABZ (1uM) for 2 h (n = 3 biological replicates). Results are presented as mean ± SD. P values were calculated using a two-sided, two-way ANOVA with Tukey’s multiple comparisons test. All results presented in this figure are representative from 3 independent experiments with similar results.

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Extended Data Fig. 8 Gating strategy for flow cytometry analysis of human PBMCs.

a, PBMCs from three donors stimulated with mock or cGAMP were stained with antibodies against p65 pS536, STING pS358, and pS366, and analyzed by flow cytometry. The diagram illustrates the gating strategy to select the populations of interest. b, c, PBMCs from three SAVI patients and two healthy controls were stained with antibodies against p65 pS536, STING pS358, and pS366, and analyzed by flow cytometry. The diagram illustrates the gating strategy to select the populations of interest.

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Extended Data Fig. 9 Functional and biochemical analysis of phosphomimetic STING C-terminal tail mutants.

a, NF-κB/ISRE reporter assay performed in HEK293T cells expressing STING-WT/S358D/S366D/S2D and transfected with NF-κB/ISRE promoter luciferase reporter and β-actin Renilla reporter (n = 3). Results are presented as mean ± SD. P values were calculated using a two-sided, one-way ANOVA with Tukey’s multiple comparisons test. b, V5 immunoprecipitation assay performed in HEK293T cells expressing different STING mutations and V5-IRF3. The precipitates were immunoblotted with anti-STING antibodies. c, SDS-PAGE for verification of peptides purification. d, Chromatogram of size exclusion chromatography of phospho-STING CTT/S358A/S366A peptide. e, Surface Plasmon Resonance (SPR) binding studies of the phosphorylated STING C-terminal tail (pSTING CTT) WT/S358A/S366A with IRF3. All results presented in this figure are representative from 3 independent experiments with similar results.

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Extended Data Fig. 10 IRF3 mediates pSTING-ser358 but not pSTING-ser366 trafficking to Endolysosome.

a, ImageStream analysis of HEK293T cells expressing WT or mutant STINGs probed for STING and the late endosome marker Rab7 (n = 3). b, ImageStream analysis of HEK293T cells stably expressing S358D were treated with Cas9 and AAVS1 (Control), AP1G1 gRNA or AP1M1 gRNA and probed for STING and late endosome markers Rab7 and LAMP1 (n = 3). c, HaCaT cells treated with Cas9 and AAVS1 (Control), AP1G1 gRNA or AP1M1 gRNA were stimulated with 25ug/ml cGAMP for 6 h. The mRNA levels of the IL6 and CXCL10 were determined by RT-q PCR (n = 3). d, ImageStream analysis of HEK293T cells stably expressing WT, S358D or S366D STING treated with Cas9 and AAVS1 or IRF3 gRNAs and probed with late endosome marker Rab7 or LAMP1 and STING (n = 3). e, Immunoblot analysis of HaCaT cells treated with Cas9 and AAVS1 or IRF3 gRNAs and stimulated with cGAMP to determine the level of indicated proteins. Results are presented as mean ± SD (ad). P values were calculated using a two-sided, one-way ANOVA with Tukey’s multiple comparisons test (a) and Dunnett’s multiple comparisons test (bd). All results presented in this figure are representative from 3 independent experiments with similar results.

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Zhang, Bc., Pedersen, A., Reinert, L.S. et al. STING signals to NF-κB from late endolysosomal compartments using IRF3 as an adaptor. Nat Immunol 26, 1916–1930 (2025). https://doi.org/10.1038/s41590-025-02283-8

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