STING activation induces polarized cytokine secretion of IFN-β and IL-17A promoting photoreceptor death and choroidal disruption in age-related macular degeneration

Age-related macular degeneration (AMD) represents one of the therapeutic challenges of aging eye diseases. Our investigation reveals the stimulator of interferon genes (STING) pathway as an orchestrator of immune-mediated retinal degeneration, exhibiting biphasic, stage-dependent functionality-providing cytoprotection in healthy tissue but driving pathogenic inflammation during early AMD progression. Through immunohistochemical analysis of human eyes, we demonstrate stage-dependent cytoplasmic STING upregulation with parallel IFN-β activation. Using patient-derived induced pluripotent stem cells-retinal pigment epithelium (iPSC-RPE) from AMD siblings, we discovered polarized cytokine secretion: apical IFN-β triggers photoreceptor apoptosis in human retinal organoids, while basal IL-17A compromises choroidal neovascularization. The Cryba1 conditional knockout (cKO) AMD-like mouse model confirms STING-driven IL-17A expression, while Il17a knock-in mice substantiate vascular alterations. STING activation establishes a pathogenic feed-forward loop between interferons and IL-17A. Single-cell transcriptomics following AAV2-mediated IFN-β overexpression reveals metabolic and phototransduction dysregulation. Both pharmacological STING inhibition with SN-011 and genetic approaches demonstrate therapeutic rescue. Cryba1/Sting double heterozygous (dhet) mice maintain homeostatic gene expression preserving retinal architecture and function. These findings establish STING as the master regulator simultaneously controlling multiple AMD pathologies through spatially organized inflammation, transforming from protective surveillance to pathogenic driver, and identifying a unified therapeutic target with demonstrated functional rescue across multiple experimental paradigms.

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Publication Details

Journal
Cell Death and Disease
Published
2026-02-27
DOI
https://doi.org/10.1038/s41419-026-08491-w
Primary Topic
interferon and immune responses
Type
article
Field-Weighted Citation Impact
0.00

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article

STING activation induces polarized cytokine secretion of IFN-β and IL-17A promoting photoreceptor death and choroidal disruption in age-related macular degeneration

Gabriella Widmer, Oksana Kutsyr, Miguel Flores‐Bellver, Peter D. Westenskow et al.
Cell Death and Disease
interferon and immune responses
article

STING activation induces polarized cytokine secretion of IFN-β and IL-17A promoting photoreceptor death and choroidal disruption in age-related macular degeneration

Gabriella Widmer, Oksana Kutsyr, Miguel Flores‐Bellver, Peter D. Westenskow, Srinivas R. Sripathi, Jakob Arnold, Pamela Strassburger, Janis Koester, Stacey Hose, Sabine Grüner, Mirjana Lazendic, Vishnu Suresh Babu, Martin Ebeling, Debasish Sinha, Puja Dutta, Eleonora M. Lad, Alan D. Proia, Sonali Singh, Sayan Ghosh, Derrick Feenstra, Pooja Gautam, Sridhar Bammidi, Chao Huang, Karl G. Csaky
article en

Abstract

Age-related macular degeneration (AMD) represents one of the therapeutic challenges of aging eye diseases. Our investigation reveals the stimulator of interferon genes (STING) pathway as an orchestrator of immune-mediated retinal degeneration, exhibiting biphasic, stage-dependent functionality-providing cytoprotection in healthy tissue but driving pathogenic inflammation during early AMD progression. Through immunohistochemical analysis of human eyes, we demonstrate stage-dependent cytoplasmic STING upregulation with parallel IFN-β activation. Using patient-derived induced pluripotent stem cells-retinal pigment epithelium (iPSC-RPE) from AMD siblings, we discovered polarized cytokine secretion: apical IFN-β triggers photoreceptor apoptosis in human retinal organoids, while basal IL-17A compromises choroidal neovascularization. The Cryba1 conditional knockout (cKO) AMD-like mouse model confirms STING-driven IL-17A expression, while Il17a knock-in mice substantiate vascular alterations. STING activation establishes a pathogenic feed-forward loop between interferons and IL-17A. Single-cell transcriptomics following AAV2-mediated IFN-β overexpression reveals metabolic and phototransduction dysregulation. Both pharmacological STING inhibition with SN-011 and genetic approaches demonstrate therapeutic rescue. Cryba1/Sting double heterozygous (dhet) mice maintain homeostatic gene expression preserving retinal architecture and function. These findings establish STING as the master regulator simultaneously controlling multiple AMD pathologies through spatially organized inflammation, transforming from protective surveillance to pathogenic driver, and identifying a unified therapeutic target with demonstrated functional rescue across multiple experimental paradigms.

Cell Death and DiseaseVol. 17(1)
Roche (Switzerland) (CH), Campbell University (US), Johns Hopkins University (US), Johns Hopkins Medicine (US), Duke Medical Center (US), Retina Foundation of the Southwest (US), Duke University Hospital (US), University of Colorado Anschutz Medical Campus (US), The University of Texas Southwestern Medical Center (US)
Research to Prevent Blindness, BrightFocus Foundation, Johns Hopkins University, Edward N. and Della L. Thome Memorial Foundation, Maryland Stem Cell Research Fund, Colorado Office of Economic Development and International Trade, National Institutes of Health
Good health and well-being
Openalex Percentile: Top 40%
interferon and immune responses
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