Repeated TLR9 stimulation results in macrophage activation syndrome-like disease in mice.

No abstract is available for this paper.

• Publication year

  2011

• Venue

  Journal of Clinical Investigation

• Publication date

  2011-06-01

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1172/JCI43157PMID 21576823PMCID PMC3104738
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• Gene expression profiling of peripheral blood mononuclear cells from children with active hemophagocytic lymphohistiocytosis.

  2011cited by this paper
• Regulation of autoreactive B cell responses to endogenous TLR ligands

  2010cited by this paper
• Normal development of mice deficient in beta 2M, MHC class I proteins, and CD8+ T cells. 1990.

  2010cited by this paper
• Macrophage activation syndrome in juvenile systemic lupus erythematosus: a multinational multicenter study of thirty-eight patients.

  2009cited by this paper
• Association study of a single nucleotide polymorphism in the exon 2 region of toll-like receptor 9 (TLR9) gene with susceptibility to systemic lupus erythematosus among Chinese

  2009cited by this paper
• Neutralization of IFNγ defeats haemophagocytosis in LCMV-infected perforin- and Rab27a-deficient mice

  2009cited by this paper
• HEMOPHAGOCYTOSIS: The Cause of Anemia and Thrombocytopenia in Congenital Syphilis

  2009cited by this paper
• Pathogenesis of multi-organic failure in autoimmune diseases.

  2009cited by this paper
• Secondary hemophagocytic lymphohistiocytosis and severe sepsis/ systemic inflammatory response syndrome/multiorgan dysfunction syndrome/macrophage activation syndrome share common intermediate phenotypes on a spectrum of inflammation.

  2009cited by this paper
• Early diagnostic and prognostic significance of a specific Th1/Th2 cytokine pattern in children with haemophagocytic syndrome

  2008cited by this paper
• The role of the initial bone marrow aspirate in the diagnosis of hemophagocytic lymphohistiocytosis

  2008cited by this paper
• TLR9 Contributes to Antiviral Immunity during Gammaherpesvirus Infection1

  2008cited by this paper
• Jinx, an MCMV susceptibility phenotype caused by disruption of Unc13d: a mouse model of type 3 familial hemophagocytic lymphohistiocytosis

  2008cited by this paper
• A Griscelli syndrome type 2 murine model of hemophagocytic lymphohistiocytosis (HLH)

  2008influential reference
• The immunology of sepsis

  2008cited by this paper
• Engagement of TLR signaling as adjuvant: towards smarter vaccine and beyond.

  2008cited by this paper
• HLH‐2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis

  2007cited by this paper
• The diagnostic significance of soluble CD163 and soluble interleukin-2 receptor alpha-chain in macrophage activation syndrome and untreated new-onset systemic juvenile idiopathic arthritis.

  2007cited by this paper
• Gene expression profiling of peripheral blood from patients with untreated new-onset systemic juvenile idiopathic arthritis reveals molecular heterogeneity that may predict macrophage activation syndrome.

  2007cited by this paper
• The vIL-10 gene of the Epstein-Barr virus (EBV) is conserved in a stable manner except for a few point mutations in various EBV isolates

  2007influential reference
• DC ablation in mice: promises, pitfalls, and challenges.

  2007cited by this paper
• Occult macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis.

  2007cited by this paper
• Notch–RBP-J signaling controls the homeostasis of CD8− dendritic cells in the spleen

  2007cited by this paper
• Phenotypic and functional profiling of human proinflammatory type‐1 and anti‐inflammatory type‐2 macrophages in response to microbial antigens and IFN‐γ‐ and CD40L‐mediated costimulation

  2006cited by this paper
• Cytokine storm in a phase 1 trial of the anti-CD28 monoclonal antibody TGN1412.

  2006cited by this paper
• Constitutive Endocytosis of CD163 Mediates Hemoglobin-Heme Uptake and Determines the Noninflammatory and Protective Transcriptional Response of Macrophages to Hemoglobin

  2006cited by this paper
• Role of TLR9 in Anti-Nucleosome and Anti-DNA Antibody Production in lpr Mutation-Induced Murine Lupus1

  2006cited by this paper
• Toll-like receptor 7 and TLR9 dictate autoantibody specificity and have opposing inflammatory and regulatory roles in a murine model of lupus.

  2006cited by this paper
• CpG-induced tyrosine phosphorylation occurs via a TLR9-independent mechanism and is required for cytokine secretion

  2006cited by this paper
• Lymphoid follicle destruction and immunosuppression after repeated CpG oligodeoxynucleotide administration

  2004influential reference
• An animal model of hemophagocytic lymphohistiocytosis (HLH): CD8+ T cells and interferon gamma are essential for the disorder.

  2004influential reference
• Constitutive Cytokine mRNAs Mark Natural Killer (NK) and NK T Cells Poised for Rapid Effector Function

  2003influential reference
• Quantitative Analysis of Cell-free Epstein-Barr Virus Genome Copy Number in Patients with EBV-associated Hemophagocytic Lymphohistiocytosis

  2002cited by this paper
• Defective LPS signaling in C3H/HeJ and C57BL/10ScCr mice: mutations in Tlr4 gene.

  1998cited by this paper
• Thrombocytopenia in the sepsis syndrome: role of hemophagocytosis and macrophage colony-stimulating factor.

  1997cited by this paper
• Cytokine production regulating Th1 and Th2 cytokines in hemophagocytic lymphohistiocytosis.

  1997cited by this paper
• Defective lymphoid development in mice lacking expression of the common cytokine receptor gamma chain.

  1995cited by this paper
• CpG motifs in bacterial DNA trigger direct B-cell activation

  1995cited by this paper
• Class I dependence of the development of CD4+ CD8- NK1.1+ thymocytes

  1994cited by this paper
• Hypercytokinemia in Hemophagocytic Syndrome

  1993cited by this paper
• Reactive hemophagocytic syndrome--a clinicopathologic study of 40 patients in an Oriental population.

  1992cited by this paper
• Normal development of mice deficient in beta 2M, MHC class I proteins, and CD8+ T cells.

  1990cited by this paper
• Histiocytic Hemophagocytosis in the Bone Marrow in Children with Sepsis and Disseminated Intravascular Coagulation

  1989cited by this paper
• Shock and tissue injury induced by recombinant human cachectin.

  1986cited by this paper
• Evidence for intravascular coagulation in systemic onset, but not polyarticular, juvenile rheumatoid arthritis.

  1985cited by this paper
• Consumption coagulopathy associated with systemic juvenile rheumatoid arthritis.

  1983cited by this paper
• Disseminated intravascular coagulation complicating systemic juvenile chronic arthritis (”Still's disease”)

  1983cited by this paper
• Still's Disease

  1969cited by this paper

• Leveraging macrophage plasticity for precision-targeted tumor immunotherapy.

  2026cites this paper
• A critical look at animal and cellular models in autoinflammatory diseases.

  2026cites this paper
• Monocyte/macrophages may exert prominent roles in macrophage activation syndrome

  2025cites this paper
• Promising Targeted Therapies for Hemophagocytic Lymphohistiocytosis: A Translational Perspective Based on Immunopathology

  2025cites this paper
• Upregulation of Fcγ Receptor IV on Activated Monocytes and Macrophages Causes Nonspecific Binding of the PK136 Anti‐NK1.1 Antibody in Murine Models of Toll‐Like Receptor‐Induced Inflammation

  2025cites this paper
• Enantiomer-dependent and modification-free DNA matrix as an adjuvant for subunit vaccines against SARS-CoV-2 or pneumococcal infections

  2025cites this paper
• Structural immunotherapy: Harnessing chemical design to build powerful next-generation therapeutics

  2025cites this paper
• Histiocyte Society blueprint for hemophagocytic lymphohistiocytosis research: deciphering underlying disease mechanisms to optimize diagnosis and therapy

  2025cites this paper
• The clinical features and outcomes of aggressive large B cell lymphoma with concomitant hemophagocytic lymphohistiocytosis at diagnosis

  2025cites this paper
• The Pathophysiology of Hemophagocytic lympho-histiocytosis (HLH) syndrome and insights from animal models.

  2025cites this paper
• Development of anti-murine IL-18 binding protein antibodies to stimulate IL-18 bioactivity.

  2025cites this paper
• Impact of Interleukin‐1 Blockade on the Development of Macrophage Activation Syndrome in Still Disease: Incidence and Diagnostic Validity of the EULAR/ACR/PRINTO 2016 MAS Classification Criteria

  2025cites this paper
• Ruxolitinib targets JAK-STAT signaling to modulate neutrophil activation in refractory macrophage activation syndrome

  2025cites this paper
• Adjuvanted RNA Origami—A Tunable Peptide Assembly Platform for Constructing Cancer Nanovaccines

  2025cites this paper
• Turmeric and Eucalyptus Aqueous Extracts Reduced Inflammation in Macrophage Activation Syndrome (MAS) Mouse Model

  2025cites this paper
• TLR9 decreases FOXO3 expression to prevent excessive inflammation in macrophage activation syndrome

  2025cites this paper
• Weal and woe of interleukin-18 in the T cell therapy of cancer

  2025cites this paper
• Clinical and Cytokine Features of Immune Effector Cell-Associated Hemophagocytic Lymphohistiocytosis-Like Syndrome.

  2025cites this paper
• Breaking the cycle: PRMTs and secondary HLH relief

  2025cites this paper
• Wolcott-Rallison Syndrome - crosstalk between PERK- EIF2A and type II interferon signaling.

  2025cites this paper
• PRMT5 inhibition reduces hyperinflammation in a murine model of secondary hemophagocytic lymphohistiocytosis

  2025cites this paper
• Neonatal Sepsis Caused by Human Parechovirus Type A3 With Marked Hyperferritinemia: A Case Report

  2025cites this paper
• STAT1 drives the immune landscape of murine Toll-like receptor 9-induced liver inflammation

  2025cites this paper
• Fatal Cytokine Collision: HLH–AIHA in Advanced AIDS—Case Report and Literature Review

  2025cites this paper
• Macrophage Activation Syndrome: Not Just for Rheumatologists Anymore.

  2025cites this paper
• T-ing up the storm: pathogenic cycling lymphocytes in the biology of macrophage activation syndrome

  2025cites this paper
• Anti-Interferon-γ Therapy for Cytokine Storm Syndromes.

  2024cites this paper
• Cytokine Storm Syndrome Associated with Systemic Juvenile Idiopathic Arthritis.

  2024cites this paper
• Cell volume tunes macrophage innate inflammatory responses through promoting type I interferon signalling

  2024cites this paper
• Mitochondria Activity and CXCR4 Collaboratively Promote the Differentiation of CD11c+ B Cells Induced by TLR9 in Lupus

  2024cites this paper
• Infectious Triggers of Cytokine Storm Syndromes: Herpes Virus Family (Non-EBV).

  2024cites this paper
• CD8+ T Cell Biology in Cytokine Storm Syndromes.

  2024cites this paper
• Kawasaki Disease-Associated Cytokine Storm Syndrome.

  2024cites this paper
• Monocytic Phagocytes in the Immunopathogenesis of Cytokine Storm Syndromes.

  2024cites this paper
• JAK Inhibitors in Cytokine Storm Syndromes.

  2024cites this paper
• Comprehensive mapping of immune perturbations associated with secondary hemophagocytic lymphohistiocytosis.

  2024cites this paper
• Immunopathology of and potential therapeutics for secondary hemophagocytic lymphohistiocytosis/macrophage activation syndrome: a translational perspective

  2024cites this paper
• Immunology of Cytokine Storm Syndromes: Natural Killer Cells.

  2024cites this paper
• The NLRP3 inflammasome is essential for IL-18 production in a murine model of macrophage activation syndrome

  2024cites this paper
• Pyruvate Kinase M2 Nuclear Translocation Regulate Ferroptosis-Associated Acute Lung Injury in Cytokine Storm

  2024cites this paper
• Infectious diseases evaluation of the child with suspected hemophagocytic lymphohistiocytosis.

  2024cites this paper
• History of Hemophagocytic Lymphohistiocytosis.

  2024cites this paper
• Identification of miRNAs that target Fcγ receptor-mediated phagocytosis during macrophage activation syndrome

  2024cites this paper
• Delayed macrophage targeting by clodronate liposomes worsens the progression of cytokine storm syndrome

  2024cites this paper
• Cytokines in Cytokine Storm Syndrome.

  2024cites this paper
• Zhilong Huoxue Tongyu Capsule regulates the macrophage polarization and inflammatory response via the let-7i/TLR9/MyD88 signaling pathway.

  2024cites this paper
• The path ahead for understanding Toll-like receptors-driven systemic autoimmunity.

  2024cites this paper
• The History of Macrophage Activation Syndrome in Autoimmune Diseases.

  2024cites this paper
• Cytokine Storm and Sepsis-Induced Multiple Organ Dysfunction Syndrome.

  2024cites this paper
• The NLRP3 inflammasome is essential for IL-18 production in a murine model of macrophage activation syndrome

  2024influential citation
• Differential Effects of JAK1 vs. JAK2 Inhibition in Mouse Models of Hemophagocytic Lymphohistiocytosis.

  2024cites this paper
• Macrophage Activation Syndrome

  2024cites this paper
• The Protective Role of Transcription Factor Nrf2 in Murine Macrophage Activation Syndrome.

  2024cites this paper
• Case report: A cyclic neutropenia patient with ELANE mutation accompanied by hemophagocytic lymphohistiocytosis

  2024cites this paper
• Liver type 1 innate lymphoid cells undergo apoptosis in murine models of macrophage activation syndrome and are dispensable for disease

  2024influential citation
• Murine Models of Secondary Cytokine Storm Syndromes.

  2024cites this paper
• Heterogeneity of macrophage activation syndrome and treatment progression

  2024cites this paper
• Development of Preliminary Criteria of Macrophage Activation Syndrome in Multisystem Inflammatory Syndrome Associated with COVID-19 in Children

  2024cites this paper
• Inflammation, immunity and biomarkers in procoagulant responses of critically ill patients.

  2024cites this paper
• Hemophagocytic Syndrome and COVID-19: A Comprehensive Review

  2023cites this paper
• A review of the pleiotropic actions of the IFN-inducible CXC chemokine receptor 3 ligands in the synovial microenvironment

  2023cites this paper
• Nucleic acid nanostructures for in vivo applications: The influence of morphology on biological fate.

  2023cites this paper
• Mouse models of systemic juvenile idiopathic arthritis and macrophage activation syndrome

  2023cites this paper
• The Multifaceted Immunology of Cytokine Storm Syndrome.

  2023cites this paper
• The 4th NextGen therapies of SJIA and MAS, part 4: it is time for IL-18 based trials in systemic juvenile idiopathic arthritis?

  2023cites this paper
• Complementary HLH susceptibility factors converge on CD8 T-cell hyperactivation

  2023cites this paper
• Liposomal Delivery of an Immunostimulatory CpG Induces Robust Antitumor Immunity and Long‐Term Immune Memory by Reprogramming Tumor‐Associated Macrophages

  2023cites this paper
• Electroacupuncture Alleviates Lung Injury in CpG1826-Challenged Mice via Modulating CD39-NLRP3 Pathway

  2023cites this paper
• Constitutively active autophagy in macrophages dampens inflammation through metabolic and post-transcriptional regulation of cytokine production.

  2023cites this paper
• The hyperinflammatory spectrum: from defects in cytotoxicity to cytokine control

  2023cites this paper
• Distinct roles of IL-18 and IL-1β in murine model of macrophage activation syndrome.

  2023cites this paper
• IL-18 Binding Protein–Producing Cells Attenuate Anemia in Murine Macrophage Activation Syndrome

  2023cites this paper
• CXCL9 inhibition does not ameliorate disease in murine models of both primary and secondary hemophagocytic lymphohistiocytosis

  2023cites this paper
• Immune Mechanisms in Inflammatory Anemia.

  2022cites this paper
• An Evidence-Based Guideline Improves Outcomes for Patients With Hemophagocytic Lymphohistiocytosis and Macrophage Activation Syndrome

  2022cites this paper
• Genetic and pharmacological targeting of GSDMD ameliorates systemic inflammation in macrophage activation syndrome.

  2022cites this paper
• Cytokine nanosponges suppressing overactive macrophages and dampening systematic cytokine storm for the treatment of hemophagocytic lymphohistiocytosis

  2022influential citation
• Pathogenic roles and diagnostic utility of interleukin-18 in autoinflammatory diseases

  2022cites this paper
• mTORC1 links pathology in experimental models of Still’s disease and macrophage activation syndrome

  2022cites this paper
• Protective role of TLR9‐induced macrophage/microglia phagocytosis after experimental intracerebral hemorrhage in mice

  2022cites this paper
• Update in Hyperferritinemic Syndromes: Recognition and Management - A Scoping Review

  2022cites this paper
• Potential Therapeutic Role of Mesenchymal-Derived Stem Cells as an Alternative Therapy to Combat COVID-19 through Cytokines Storm

  2022cites this paper
• Interleukin-18: a biomarker with therapeutic potential in adult-onset Still’s disease

  2022cites this paper
• The clinical heterogeneity of adult onset Still's disease may underlie different pathogenic mechanisms. Implications for a personalised therapeutic management of these patients.

  2022cites this paper
• Expansion of CD4dimCD8+T cells characterizes macrophage activation syndrome and other secondary HLH.

  2022cites this paper
• Macrophage activation syndrome in adults: Characteristics, outcomes, and therapeutic effectiveness of etoposide-based regimen

  2022cites this paper
• Role of Distinct Macrophage Populations in the Development of Heart Failure in Macrophage Activation Syndrome

  2022cites this paper
• Cytokine storm and targeted therapy in hemophagocytic lymphohistiocytosis

  2022cites this paper
• The Role of Interleukin 18/Interleukin 18-Binding Protein in Adult-Onset Still’s Disease and Systemic Juvenile Idiopathic Arthritis

  2022cites this paper
• T cell activation and IFNγ modulate organ dysfunction in LPS‐mediated inflammation

  2022cites this paper
• Sepsis with liver dysfunction and coagulopathy predicts an inflammatory pattern of macrophage activation

  2022cites this paper
• Looking into the IL-1 of the storm: are inflammasomes the link between immunothrombosis and hyperinflammation in cytokine storm syndromes?

  2022cites this paper
• Hemophagocytic lymphohistiocytosis in adults: A key issue in the COVID-19 era

  2021cites this paper
• Macrophage activation syndrome in COVID-19

  2021cites this paper
• Adverse Events and Side Effects of Chimeric Antigen Receptor (CAR) T Cell Therapy in Patients with Hematologic Malignancies

  2021cites this paper
• Targeting interferon-γ in hyperinflammation: opportunities and challenges

  2021cites this paper
• IFN-γ is essential for alveolar macrophage–driven pulmonary inflammation in macrophage activation syndrome

  2021influential citation
• Interfering with Interferons: targeting the JAK-STAT pathway in complications of systemic juvenile idiopathic arthritis (SJIA).

  2021cites this paper
• Case Report: Rapid Recognition and Immune Modulation of Secondary HLH Due to Disseminated HSV Infection

  2021cites this paper
• Macrophage activation syndrome: A diagnostic challenge (Review)

  2021cites this paper