Scandinavian Journal of Immunology最新文献_第4页

UNC13D in Familial Hemophagocytic Lymphohistiocytosis and Beyond: Functional Mechanisms, Genetic Variants, Multisystem Disease Spectrum and Clinical Implications. 家族性噬血细胞淋巴组织细胞病的UNC13D：功能机制，遗传变异，多系统疾病谱系和临床意义。

IF 2.4 4区医学

Scandinavian Journal of Immunology Pub Date : 2026-05-01 DOI: 10.1111/sji.70117

Ziqian Wang, Chong Chen, Zhaojun Duan, Chong Wei, Wei Zhang, Yunping Luo, Daobin Zhou

{"title":"UNC13D in Familial Hemophagocytic Lymphohistiocytosis and Beyond: Functional Mechanisms, Genetic Variants, Multisystem Disease Spectrum and Clinical Implications.","authors":"Ziqian Wang, Chong Chen, Zhaojun Duan, Chong Wei, Wei Zhang, Yunping Luo, Daobin Zhou","doi":"10.1111/sji.70117","DOIUrl":"10.1111/sji.70117","url":null,"abstract":"The UNC13D gene encodes Munc13-4, a key regulator of cytotoxic granule exocytosis in effector immune cells, enabling the release of perforin and granzymes that are essential for cytotoxic function and immune surveillance. Loss-of-function mutations in UNC13D result in immune dysregulation syndromes, most notably familial hemophagocytic lymphohistiocytosis (fHLH, also referred to as FHL). This review provides a comprehensive overview of UNC13D, including its structural characteristics, biological functions, and spectrum of pathogenic variants. We summarize the mechanistic roles of Munc13-4 in granule-mediated cytotoxicity and examine the clinical correlations between UNC13D mutations and fHLH type 3 (FHL3). Furthermore, we discuss emerging evidence linking UNC13D dysfunction to a broader range of diseases, highlighting its clinical relevance and potential as both a diagnostic biomarker and therapeutic target. Overall, this review aims to bridge the gap between molecular mechanisms and clinical translation in UNC13D-related disorders.","PeriodicalId":21493,"journal":{"name":"Scandinavian Journal of Immunology","volume":"103 5","pages":"e70117"},"PeriodicalIF":2.4,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147779879","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Molecular Mechanisms of NETs and Platelet Interactions and Their Regulatory Functions in Sepsis. 脓毒症中NETs与血小板相互作用的分子机制及其调控功能。

IF 2.4 4区医学

Scandinavian Journal of Immunology Pub Date : 2026-05-01 DOI: 10.1111/sji.70120

Zhongji Han, Jing Xu, Maokui Yue

引用次数: 0

COVID-19 and Pregnancy: Key Findings. COVID-19与妊娠：主要发现。

IF 2.4 4区医学

Scandinavian Journal of Immunology Pub Date : 2026-04-01 DOI: 10.1111/sji.70109

Gabrielle Gimenes Lima, Ana Flávia Segati, Giovanna Santos Oliveira, Nicoly Simões de Melo, Túlio Nakazato da Cunha, Elizabeth De Gaspari

引用次数: 0

RETRACTION: Restoration of Impaired Free Radical Generation and Proinflammatory Cytokines by MCP-1 in Mycobacterial Pathogenesis. 撤回：MCP-1在分枝杆菌发病机制中修复受损的自由基生成和促炎细胞因子。

IF 1.6 4区医学

Scandinavian Journal of Immunology Pub Date : 2026-04-01 DOI: 10.1111/sji.70111

引用次数: 0

Decoding NK Cell Subset Dysregulation in SARS-CoV-2 Infection: Phenotypic, Functional, and Transcriptomic Insights Into COVID-19 Pathogenesis. 解码SARS-CoV-2感染中NK细胞亚群失调：对COVID-19发病机制的表型、功能和转录组学见解

IF 2.4 4区医学

Scandinavian Journal of Immunology Pub Date : 2026-04-01 DOI: 10.1111/sji.70115

Marlyn D Sepúlveda, Walter D Cardona Maya, Wildeman Zapata-Builes, Paula A Velilla

{"title":"Decoding NK Cell Subset Dysregulation in SARS-CoV-2 Infection: Phenotypic, Functional, and Transcriptomic Insights Into COVID-19 Pathogenesis.","authors":"Marlyn D Sepúlveda, Walter D Cardona Maya, Wildeman Zapata-Builes, Paula A Velilla","doi":"10.1111/sji.70115","DOIUrl":"10.1111/sji.70115","url":null,"abstract":"Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection significantly affects innate immune responses, particularly those of natural killer (NK) cells, which play an important role in antiviral defence. This review combines phenotypic, functional, and transcriptomic findings to explain the disruption of NK cell subsets in COVID-19. Flow cytometry studies have shown generalised lymphopenia and a significant reduction in the CD56bright and CD56dimCD16+ subsets. This change is linked to an increase in the CD56dimCD16- and CD56-CD16+ populations, which correlate with disease severity. At the molecular level, there is an imbalance between activating and inhibitory receptors. This includes increases in NKG2A, PD-1, and LAG-3, along with decreases in NKp30, NKp46, and NKG2D. These findings are consistent with an exhausted phenotype and weakened cytotoxicity. Single-cell RNA sequencing (scRNA-seq) studies have identified an increase in proliferative, cytotoxic, and platelet-associated CD56dim NK subpopulations in severe cases and a reduction of CD56bright cells. Transcriptomic profiling showed that upregulation of interferon-stimulated genes (ISGs) and inflammatory pathways driven by STAT1/3, NF-κB activation, and transforming growth factor-beta (TGF-β) signalling suppresses NK effector functions. Collectively, these findings suggest a model in which progressive NK cell dysfunction may be associated with the immunopathogenesis of COVID-19. The integration of multi-omic and phenotypic approaches provides a comprehensive view of NK cell responses to SARS-CoV-2 and suggests potential biomarkers and therapeutic targets to restore NK cell antiviral activity.","PeriodicalId":21493,"journal":{"name":"Scandinavian Journal of Immunology","volume":"103 4","pages":"e70115"},"PeriodicalIF":2.4,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147691995","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Murine Intestinal Neonatal and Adult Dendritic Cell Subset Migration and Activation Characteristics in Response to Toll-Like Receptor Stimulation Through MyD88. 通过MyD88响应toll样受体刺激的小鼠肠道新生和成年树突状细胞亚群迁移和激活特性

IF 2.4 4区医学

Scandinavian Journal of Immunology Pub Date : 2026-04-01 DOI: 10.1111/sji.70114

Konjit Getachew Muleta, Signe Holst, Agnès Garcias López, Sayoni Chakraborty, Clement da Silva, Adam F Cunningham, Isabel Ulmert, Joy Nakawesi, Julia Hütter, Katharina Lahl

{"title":"Murine Intestinal Neonatal and Adult Dendritic Cell Subset Migration and Activation Characteristics in Response to Toll-Like Receptor Stimulation Through MyD88.","authors":"Konjit Getachew Muleta, Signe Holst, Agnès Garcias López, Sayoni Chakraborty, Clement da Silva, Adam F Cunningham, Isabel Ulmert, Joy Nakawesi, Julia Hütter, Katharina Lahl","doi":"10.1111/sji.70114","DOIUrl":"10.1111/sji.70114","url":null,"abstract":"The initiation of adaptive immunity requires antigen presentation in immune inductive sites. Classical dendritic cells (cDC) sense peripheral immune response inducing cues and migrate to the tissue-draining lymph nodes upon engagement, where they present acquired antigen to cognate T cells. Vaccines include adjuvants, which enable cDC activation and migration, both essential steps in the cascade leading to adaptive immunity. We here show that the expression of MyD88, the adapter molecule downstream of most toll-like receptors (TLR), is essential for the migration of cDCs in response to several stimuli and that its restricted expression to the TLR-sensing cDC subset is sufficient for the migration of that subset. As efficacious oral vaccines are primarily needed for use in newborns, we further quantify and characterise neonatal intestinal cDCs in five-day-old mice and show that they follow the same rules and patterns as we identified for cDCs in the adult intestines. Together, our data suggest strong conservation of immune-inducing cues across ages and provide fundamental knowledge aiding adjuvant choice for neonatal vaccination strategies.","PeriodicalId":21493,"journal":{"name":"Scandinavian Journal of Immunology","volume":"103 4","pages":"e70114"},"PeriodicalIF":2.4,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13080077/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147692016","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Itaconate as a Potent Regulator of Neutrophil Responses in Host Defence and Inflammation. 衣康酸作为宿主防御和炎症中性粒细胞反应的有效调节剂。

IF 2.4 4区医学

Scandinavian Journal of Immunology Pub Date : 2026-04-01 DOI: 10.1111/sji.70112

Chunying Cheng, Zufeng Cheng, Xinran Ye, Siwei Wang, Changqing Yan, Yiran Chen, Jie Li, Rundong Wu, Aihua Lei

{"title":"Itaconate as a Potent Regulator of Neutrophil Responses in Host Defence and Inflammation.","authors":"Chunying Cheng, Zufeng Cheng, Xinran Ye, Siwei Wang, Changqing Yan, Yiran Chen, Jie Li, Rundong Wu, Aihua Lei","doi":"10.1111/sji.70112","DOIUrl":"10.1111/sji.70112","url":null,"abstract":"Itaconate, derived from cis-aconitate decarboxylation by immune-responsive gene 1 (IRG1; also called cis-aconitate decarboxylase 1, ACOD1), is an intermediate metabolite of the tricarboxylic acid (TCA) cycle in the mitochondria. The production of itaconate in myeloid cells is rapidly increased to high levels in pathological conditions, such as infection and cancer. It is well known that itaconate plays an essential role in regulating macrophage-mediated inflammation and immune response through multiple mechanisms, such as regulating signal transduction and protein modification. As the first responders upon infections and injuries, neutrophils contribute to pathogen clearance and inflammation by several mechanisms, such as phagocytosis, producing reactive oxygen species (ROS), and forming neutrophil extracellular traps (NETs). Increasing evidence shows that neutrophils can also produce itaconate, which in turn modulates neutrophil activation, thereby affecting the elimination of pathogens, the resolution of inflammation, and tumour progression. In this review, we summarize the recent advancements in understanding the effects of endogenous itaconate and its derivatives on neutrophil responses, with a focus on the underlying mechanisms and potential therapeutic applications in infectious and inflammatory diseases.","PeriodicalId":21493,"journal":{"name":"Scandinavian Journal of Immunology","volume":"103 4","pages":"e70112"},"PeriodicalIF":2.4,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147594408","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Clinical Manifestations and Immunologic Phenotype in Patients With RAG1/2 Mutations: A Single Centre Experience and Description of A Novel Mutation. RAG1/2突变患者的临床表现和免疫表型：单一中心经验和新突变的描述。

IF 2.4 4区医学

Scandinavian Journal of Immunology Pub Date : 2026-04-01 DOI: 10.1111/sji.70116

Işılay Turan, Selami Ulaş, Sezin Naiboğlu, Pınar Gökmirza, Serdar Al, Mehmet Halil Çeliksoy, Çiğdem Aydoğmuş

引用次数: 0

Allergic Diseases in Common Variable Immunodeficiency: A Prospective Cross-Sectional Study on Prevalence and Allergy Biomarkers of Clinical Phenotypes. 共同可变免疫缺陷的过敏性疾病：临床表型的流行和过敏生物标志物的前瞻性横断面研究。

IF 2.4 4区医学

Scandinavian Journal of Immunology Pub Date : 2026-03-01 DOI: 10.1111/sji.70108

Maria Giovanna Danieli, Ilaria Claudi, Stefania Auria, Matteo Martini, Elena Buti, Silvia Brunetto, Gianluca Moroncini, Paola Lucia Minciullo, Sebastiano Gangemi, Maria Beatrice Bilò

{"title":"Allergic Diseases in Common Variable Immunodeficiency: A Prospective Cross-Sectional Study on Prevalence and Allergy Biomarkers of Clinical Phenotypes.","authors":"Maria Giovanna Danieli, Ilaria Claudi, Stefania Auria, Matteo Martini, Elena Buti, Silvia Brunetto, Gianluca Moroncini, Paola Lucia Minciullo, Sebastiano Gangemi, Maria Beatrice Bilò","doi":"10.1111/sji.70108","DOIUrl":"10.1111/sji.70108","url":null,"abstract":"The link between allergic conditions and common variable immunodeficiency (CVID) is still unclear. Only a few studies suggest allergic diseases are more prevalent in CVID patients than in the general population, and the role of IgE remains poorly defined. This study aims to evaluate the prevalence of allergic conditions in CVID and the role of serum IgE and IgA levels. This prospective, cross-sectional, case-control study enrolled Italian adult CVID patients to investigate allergic conditions' frequency and relationships between IgE, IgA, and clinical phenotypes. Analyses of diagnostic/prognostic accuracy were performed with ROC curves. We documented an allergic disease in 26.6% of 60 CVID patients, most commonly allergic rhinitis (56.2%) and bronchial asthma (12.5%). CVID patients with allergy had higher IgE levels (+8.9 kU/L, p = 0.006) than non-allergic ones, but lower than allergic individuals without CVID. IgE deficiency was observed in 65% of CVID patients, with a strong correlation between IgE and IgA levels (r = 0.7, p < 0.001). Low IgE (< 2.5 kU/L) and IgA levels (< 7 mg/dL) were significantly associated with lymphoproliferative (p < 0.001) and granulomatous phenotypes (p = 0.005), achieving an AUC of 94% and 81% for predicting lymphoproliferation and granulomatosis, respectively. The prevalence of allergic conditions in CVID patients is lower compared with previous studies. Low IgE levels served as a good biomarker for CVID and CVID-phenotypes. Combined serum IgE and IgA assessment improved prognostic stratification.","PeriodicalId":21493,"journal":{"name":"Scandinavian Journal of Immunology","volume":"103 3","pages":"e70108"},"PeriodicalIF":2.4,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12996437/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147475154","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

IL40: A Newly Described Cytokine With Conflicting Measurements and Detection Variability-Are There Different Forms? IL40：一种新描述的细胞因子，具有相互冲突的测量和检测变异-是否存在不同的形式？

IF 2.4 4区医学

Scandinavian Journal of Immunology Pub Date : 2026-03-01 DOI: 10.1111/sji.70105

Nora Euler, Wenqi Huang, Peter Hemmingsson, Anna Juto, Erik Hellbacher, Lars Klareskog, Vivianne Malmström, Iva Gunnarsson, Eva Baecklund, Caroline Grönwall

{"title":"IL40: A Newly Described Cytokine With Conflicting Measurements and Detection Variability-Are There Different Forms?","authors":"Nora Euler, Wenqi Huang, Peter Hemmingsson, Anna Juto, Erik Hellbacher, Lars Klareskog, Vivianne Malmström, Iva Gunnarsson, Eva Baecklund, Caroline Grönwall","doi":"10.1111/sji.70105","DOIUrl":"10.1111/sji.70105","url":null,"abstract":"Interleukin-40 (IL40) is a recently described 27 kDa cytokine encoded by C17orf99, originally suggested to play a role in B-cell biology, but its function is largely unknown. However, elevated serum levels have been reported in rheumatic diseases. Most published studies focus on IL40 measurements in serum/plasma using commercial sandwich ELISAs. Here we found large discrepancies between two IL40 ELISAs (Mybiosource and Abbexa), with Abbexa reporting significantly higher plasma levels. In our investigation, IL40 (Abbexa) was not elevated in patients with ANCA-associated vasculitis, early or established rheumatoid arthritis (RA), or RA patients who had developed B cell lymphoma (RA-L), compared to healthy donors. Yet, we found significant correlation of Abbexa IL40 levels with BAFF and APRIL. We next compared the binding of IL40 between the two commercial assays. Pre-adsorption experiments showed that the Mybiosource capture antibody bound the same target as the Abbexa capture antibody but did not detect the same IL40. Moreover, neither assay detected the reciprocal IL40 kit reference nor mammalian expressed recombinant IL40. In contrast, a Human Protein Atlas (HPA) antibody towards the unstructured C-terminal of IL40, despite being only partly validated by HPA, detected recombinant IL40 in Western blot and ELISA. We speculate that there may be different structural or modified forms of IL40. The discrepancy between the IL40 Abbexa results and the literature also highlights the difficulties in interpreting results from commercial antibodies and assays.","PeriodicalId":21493,"journal":{"name":"Scandinavian Journal of Immunology","volume":"103 3","pages":"e70105"},"PeriodicalIF":2.4,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12967690/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147378467","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0