TSC22 domain family member 3 links natural killer cells to CD8+ T cell-mediated drug hypersensitivity

Abstract Severe cutaneous adverse drug reactions (SCARs) are life-threatening diseases, which are associated with human leukocyte antigen (HLA) risk variants. However, the low positive predictive values of HLA variants suggest additional factors influence disease susceptibility. Using dapsone hypers...

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Main Authors: Lele Sun, Pengcheng Huai, Zhenzhen Wang, Qing Zhao, Yingjie Lin, Tingting Liu, Xiaotong Xue, Suiting Ao, Jiabao You, Yonghu Sun, Zihao Mi, Joshua Gardner, Paul J. Thomson, Dean J. Naisbitt, Xiaoli Meng, Jianjun Liu, Hong Liu, Furen Zhang
Format: Article
Language:English
Published: Nature Publishing Group 2025-06-01
Series:Signal Transduction and Targeted Therapy
Online Access:https://doi.org/10.1038/s41392-025-02300-0
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author Lele Sun
Pengcheng Huai
Zhenzhen Wang
Qing Zhao
Yingjie Lin
Tingting Liu
Xiaotong Xue
Suiting Ao
Jiabao You
Yonghu Sun
Zihao Mi
Joshua Gardner
Paul J. Thomson
Dean J. Naisbitt
Xiaoli Meng
Jianjun Liu
Hong Liu
Furen Zhang
author_facet Lele Sun
Pengcheng Huai
Zhenzhen Wang
Qing Zhao
Yingjie Lin
Tingting Liu
Xiaotong Xue
Suiting Ao
Jiabao You
Yonghu Sun
Zihao Mi
Joshua Gardner
Paul J. Thomson
Dean J. Naisbitt
Xiaoli Meng
Jianjun Liu
Hong Liu
Furen Zhang
author_sort Lele Sun
collection DOAJ
description Abstract Severe cutaneous adverse drug reactions (SCARs) are life-threatening diseases, which are associated with human leukocyte antigen (HLA) risk variants. However, the low positive predictive values of HLA variants suggest additional factors influence disease susceptibility. Using dapsone hypersensitivity syndrome (DHS) as a paradigm for SCARs, we show that the DHS patients harbor a sex-related global reduction in blood NK cells, contributing to the higher incidence of reactions in females. Single-cell RNA sequencing revealed a decrease in the immunoregulatory CD56 low XCL1/2 low NK cell subset and an expansion of CD56 high XCL1/2 high NK cell subsets with an effector phenotype in DHS patients compared to dapsone-tolerant individuals. Functionally, interleukin-15 superagonist-induced activation of NK cells exacerbated SCARs-like symptoms in a murine model. Mechanistically, TSC22 domain family member 3 (TSC22D3) deficiency enhanced NK cell effector function, shifting the immune response from CD4+ T cell to CD8+ T cell function. These results demonstrate that TSC22D3-regulated NK cells play a critical role in predisposing to drug hypersensitivity reactions, bridging innate and adaptive immune dysregulation in SCARs pathogenesis. Our study highlights the importance of NK cell heterogeneity and TSC22D3 in immune-mediated hypersensitivity disorders, offering potential therapeutic targets for SCARs and related conditions.
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spelling doaj-art-e5afd5eb1d7b4401a48af3af894805fe2025-08-20T02:10:38ZengNature Publishing GroupSignal Transduction and Targeted Therapy2059-36352025-06-0110111410.1038/s41392-025-02300-0TSC22 domain family member 3 links natural killer cells to CD8+ T cell-mediated drug hypersensitivityLele Sun0Pengcheng Huai1Zhenzhen Wang2Qing Zhao3Yingjie Lin4Tingting Liu5Xiaotong Xue6Suiting Ao7Jiabao You8Yonghu Sun9Zihao Mi10Joshua Gardner11Paul J. Thomson12Dean J. Naisbitt13Xiaoli Meng14Jianjun Liu15Hong Liu16Furen Zhang17Dermatology Hospital of Shandong First Medical UniversityDermatology Hospital of Shandong First Medical UniversityDermatology Hospital of Shandong First Medical UniversityDermatology Hospital of Shandong First Medical UniversityDermatology Hospital of Shandong First Medical UniversityDermatology Hospital of Shandong First Medical UniversityDermatology Hospital of Shandong First Medical UniversityDermatology Hospital of Shandong First Medical UniversityDermatology Hospital of Shandong First Medical UniversityDermatology Hospital of Shandong First Medical UniversityDermatology Hospital of Shandong First Medical UniversityMRC Centre for Drug Safety Science, Department of Pharmacology and Therapeutic, Institute of Systems, Molecular and Integrative Biology, University of LiverpoolMRC Centre for Drug Safety Science, Department of Pharmacology and Therapeutic, Institute of Systems, Molecular and Integrative Biology, University of LiverpoolMRC Centre for Drug Safety Science, Department of Pharmacology and Therapeutic, Institute of Systems, Molecular and Integrative Biology, University of LiverpoolMRC Centre for Drug Safety Science, Department of Pharmacology and Therapeutic, Institute of Systems, Molecular and Integrative Biology, University of LiverpoolLaboratory of Human Genomics, Genome Institute of Singapore (GIS), Agency for Science, Technology and Research (A*STAR), Singapore, Republic of Singapore; Yong Loo Lin School of Medicine, National University of SingaporeDermatology Hospital of Shandong First Medical UniversityDermatology Hospital of Shandong First Medical UniversityAbstract Severe cutaneous adverse drug reactions (SCARs) are life-threatening diseases, which are associated with human leukocyte antigen (HLA) risk variants. However, the low positive predictive values of HLA variants suggest additional factors influence disease susceptibility. Using dapsone hypersensitivity syndrome (DHS) as a paradigm for SCARs, we show that the DHS patients harbor a sex-related global reduction in blood NK cells, contributing to the higher incidence of reactions in females. Single-cell RNA sequencing revealed a decrease in the immunoregulatory CD56 low XCL1/2 low NK cell subset and an expansion of CD56 high XCL1/2 high NK cell subsets with an effector phenotype in DHS patients compared to dapsone-tolerant individuals. Functionally, interleukin-15 superagonist-induced activation of NK cells exacerbated SCARs-like symptoms in a murine model. Mechanistically, TSC22 domain family member 3 (TSC22D3) deficiency enhanced NK cell effector function, shifting the immune response from CD4+ T cell to CD8+ T cell function. These results demonstrate that TSC22D3-regulated NK cells play a critical role in predisposing to drug hypersensitivity reactions, bridging innate and adaptive immune dysregulation in SCARs pathogenesis. Our study highlights the importance of NK cell heterogeneity and TSC22D3 in immune-mediated hypersensitivity disorders, offering potential therapeutic targets for SCARs and related conditions.https://doi.org/10.1038/s41392-025-02300-0
spellingShingle Lele Sun
Pengcheng Huai
Zhenzhen Wang
Qing Zhao
Yingjie Lin
Tingting Liu
Xiaotong Xue
Suiting Ao
Jiabao You
Yonghu Sun
Zihao Mi
Joshua Gardner
Paul J. Thomson
Dean J. Naisbitt
Xiaoli Meng
Jianjun Liu
Hong Liu
Furen Zhang
TSC22 domain family member 3 links natural killer cells to CD8+ T cell-mediated drug hypersensitivity
Signal Transduction and Targeted Therapy
title TSC22 domain family member 3 links natural killer cells to CD8+ T cell-mediated drug hypersensitivity
title_full TSC22 domain family member 3 links natural killer cells to CD8+ T cell-mediated drug hypersensitivity
title_fullStr TSC22 domain family member 3 links natural killer cells to CD8+ T cell-mediated drug hypersensitivity
title_full_unstemmed TSC22 domain family member 3 links natural killer cells to CD8+ T cell-mediated drug hypersensitivity
title_short TSC22 domain family member 3 links natural killer cells to CD8+ T cell-mediated drug hypersensitivity
title_sort tsc22 domain family member 3 links natural killer cells to cd8 t cell mediated drug hypersensitivity
url https://doi.org/10.1038/s41392-025-02300-0
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