ArticleScience immunology2023
Encephalitis and poor neuronal death-mediated control of herpes simplex virus in human inherited RIPK3 deficiency.
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Article in Science immunology, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 35 papers.
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35citing papers in PubMed
8.1field-weighted citation impact, top 2% of its field
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Who cites it
35 citing papers in PubMed, 49 citations in OpenAlex.
- A Rare RIPK3 Variant Enhances Necroptosis and Promotes Inflammation in a Still Disease-Like Autoinflammatory Syndrome.Arthritis & rheumatology (Hoboken, N.J.) · 2026Article
- Mechanisms of regeneration and maladaptive repair in acute kidney injury.Nature reviews. Nephrology · 2026Review
- Cell death network regulation in HSV infection: immune evasion versus host defense.Apoptosis : an international journal on programmed cell death · 2026Review
- Functional amyloids as molecular switches: emerging roles in diverse signalling pathways.Biochemical Society transactions · 2026Review
- Chronic granulomatosis with persistent HSV intrathecal synthesis over three years: a rare complication of HSV encephalitis in adult.Journal of neurovirology · 2026Article
- Optimization of lytic herpes simplex virus infection in human induced pluripotent stem cell-derived cortical neurones.The Journal of general virology · 2026Article
- The immune system as a regulator of normal physiology.Frontiers in immunology · 2026Review
- Monogenic disorders of the TNF signalling pathway.Nature reviews. Rheumatology · 2026Review
- GADD45β inhibits RIPK3-mediated NF-κB activation by interfering with NEMO-RIPK1-RIPK3 interactions.Cell death discovery · 2025Article
- Human inborn errors of type I interferon-independent intrinsic immunity in nonleukocytic cells.Current opinion in immunology · 2025Review
- Oncolytic virus-induced IL-1βJournal for immunotherapy of cancer · 2025Article
- Necroptotic cell death consequences and disease relevance.Nature immunology · 2025Review
- Human inborn errors of immunity: 2024 update on the classification from the International Union of Immunological Societies Expert Committee.Journal of human immunity · 2025Article
- The immunogenetic basis of severe herpes simplex infections in neonates and children: a review.Pediatric research · 2025Review
- The monogenic landscape of human infectious diseases.The Journal of allergy and clinical immunology · 2025Review
- RIPK1 is required for ZBP1-driven necroptosis in human cells.PLoS biology · 2025Article
- Human DBR1 deficiency impairs stress granule-dependent PKR antiviral immunity.The Journal of experimental medicine · 2025Article
- Advances in cell therapy for orthopedic diseases: bridging immune modulation and regeneration.Frontiers in immunology · 2025Review
- Necroptosis in vascular cognitive impairment: mechanisms and therapeutic potential.Frontiers in aging neuroscience · 2025Review
- Role of necroptosis in pulpitis: integration of bacterial infection, immune imbalance, and oxidative stress.PeerJ · 2025Review
Corrections and comments
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Authors and funding
41 authors at 13 institutions in 6 countries.
Zhiyong LiuSt. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, Rockefeller University, New York, NY, USA.ORCID 0000-0001-6473-348X
Eduardo J Garcia ReinoSt. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, Rockefeller University, New York, NY, USA.ORCID 0000-0002-4781-3864
Oliver HarschnitzThe Center for Stem Cell Biology, Sloan Kettering Institute for Cancer Research, New York, NY, USA.ORCID 0000-0001-6496-881X
Hongyan GuoDepartment of Microbiology and Immunology, Emory Vaccine Center, Emory University, GA, USA.ORCID 0000-0002-6286-1741
Yi-Hao ChanSt. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, Rockefeller University, New York, NY, USA.ORCID 0000-0003-0329-238X
Noopur V KhobrekarThe Center for Stem Cell Biology, Sloan Kettering Institute for Cancer Research, New York, NY, USA.
Mary L HasekSt. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, Rockefeller University, New York, NY, USA.ORCID 0000-0001-8902-6431
Kerry DobbsLaboratory of Clinical Immunology and Microbiology, National Institute of Allergy and Infectious Diseases, NIH, Bethesda, MD, USA.ORCID 0000-0002-3432-3137
Darawan RinchaiSt. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, Rockefeller University, New York, NY, USA.ORCID 0000-0001-8851-7730
Marie MaternaLaboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, Necker Hospital for Sick Children, Paris, France.ORCID 0000-0003-2145-5285
Daniela MatuozzoLaboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, Necker Hospital for Sick Children, Paris, France.ORCID 0000-0001-9514-3837
Danyel LeeSt. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, Rockefeller University, New York, NY, USA.ORCID 0000-0003-3124-0245
Paul BastardSt. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, Rockefeller University, New York, NY, USA.ORCID 0000-0002-5926-8437
Jie ChenSt. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, Rockefeller University, New York, NY, USA.
Yoon Seung LeeSt. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, Rockefeller University, New York, NY, USA.ORCID 0000-0002-9988-8905
Seong K KimSchool of Medicine, Atlanta, GA, USA.
Shuxiang ZhaoSt. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, Rockefeller University, New York, NY, USA.ORCID 0000-0001-9185-6813
Param AminThe Center for Stem Cell Biology, Sloan Kettering Institute for Cancer Research, New York, NY, USA.
Lazaro LorenzoLaboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, Necker Hospital for Sick Children, Paris, France.ORCID 0000-0001-6648-8684
Yoann SeeleuthnerLaboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, Necker Hospital for Sick Children, Paris, France.
Remi ChevalierLaboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, Necker Hospital for Sick Children, Paris, France.ORCID 0000-0002-8611-1937
Laure MazzolaDepartment of Pediatrics, Hôpital Nord, Saint-Etienne, Paris, France.
Claire GayDepartment of Pediatrics, Hôpital Nord, Saint-Etienne, Paris, France.
Jean-Louis StephanDepartment of Pediatrics, Hôpital Nord, Saint-Etienne, Paris, France.
Baptiste MilisavljevicSt. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, Rockefeller University, New York, NY, USA.
Soraya BoucheritLaboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, Necker Hospital for Sick Children, Paris, France.ORCID 0000-0002-8819-7594
Flore RozenbergLaboratory of Virology, Assistance Publique-Hôpitaux de Paris (AP-HP), Cochin Hospital, Paris, France.
Rebeca Perez de DiegoLaboratory of Immunogenetics of Human Diseases, IdiPAZ Institute for Health Research, La Paz Hospital, Madrid, Spain.ORCID 0000-0001-8426-8765
Richard D DixViral Immunology Center, Department of Biology, Georgia State University, Atlanta, GA, USA.ORCID 0000-0002-7317-3549
Nico MarrResearch Branch, Sidra Medicine, Doha, Qatar.ORCID 0000-0002-1927-7072
Vivien BéziatSt. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, Rockefeller University, New York, NY, USA.ORCID 0000-0002-4020-824X
Aurelie CobatSt. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, Rockefeller University, New York, NY, USA.ORCID 0000-0001-7209-6257
Mélodie AubartLaboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, Necker Hospital for Sick Children, Paris, France.ORCID 0000-0002-4383-7930
Laurent AbelSt. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, Rockefeller University, New York, NY, USA.ORCID 0000-0001-7016-6493
Stephane ChabrierDepartment of Pediatrics, Hôpital Nord, Saint-Etienne, Paris, France.ORCID 0000-0002-6816-3266
Gregory A SmithDepartment of Microbiology-Immunology, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
Luigi D NotarangeloLaboratory of Clinical Immunology and Microbiology, National Institute of Allergy and Infectious Diseases, NIH, Bethesda, MD, USA.ORCID 0000-0002-8335-0262
Edward S MocarskiDepartment of Microbiology and Immunology, Emory Vaccine Center, Emory University, GA, USA.
Lorenz StuderThe Center for Stem Cell Biology, Sloan Kettering Institute for Cancer Research, New York, NY, USA.ORCID 0000-0003-0741-7987
Jean-Laurent CasanovaSt. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, Rockefeller University, New York, NY, USA.ORCID 0000-0002-7782-4169
Shen-Ying ZhangSt. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, Rockefeller University, New York, NY, USA.ORCID 0000-0002-9449-3672
Inserm · FRRockefeller University · USHôpital Nord · FRKettering University · USEmory Healthcare · USNational Institute of Allergy and Infectious Diseases · USEmory University · USHamad bin Khalifa University · QAHôpital Cochin · FRHospital La Paz Institute for Health Research · ESHoward Hughes Medical Institute · USLouisiana State University Health Sciences Center Shreveport · USNorthwestern University · US
Funding
X-RAY CRYSTALLOGRAPHYP30CA008748 · NCI · SLOAN-KETTERING INSTITUTE FOR CANCER RES · PI SELWYN M VICKERS · 1985 to 2026
$347.4MDeveloping, Demonstrating, and Disseminating Innovative Programs to Achieve Translational SuccessUL1TR001866 · NCATS · ROCKEFELLER UNIVERSITY · PI COLLER, BARRY, KRUEGER, JAMES G · 2016 to 2025
$40.6MCharacterization of the cellular and molecular bases of inborn errors of immunityZIAAI001222 · NIAID · NATIONAL INSTITUTE OF ALLERGY AND INFECTIOUS DISEASES · PI NOTARANGELO, LUIGI · 2017 to 2025
$15.2MStudy of the Cellular Basis of Herpes Simplex Encephalitis with hPSC-Derived CNS and PNS CellsR01NS072381 · NINDS · UT SOUTHWESTERN MEDICAL CENTER · PI Jean-Laurent Casanova, Shen-Ying Zhang · 2010 to 2026
$10.2MMonogenic basis of resistance to SARS-CoV2 and predisposition to severe COVID-19R01AI088364 · NIAID · UT SOUTHWESTERN MEDICAL CENTER · PI Jean-Laurent Casanova, Shen-Ying Zhang · 2010 to 2026
$8.7MCYTOMEGALOVIRUS DNA REPLICATION AND RECOMBINATIONR01AI020211 · NIAID · STANFORD UNIVERSITY · PI EDWARD S MOCARSKI, EDWARD S. · 1985 to 2021
$5.9MCytomegalovirus retinitis pathogenesis: Mechanisms of retinal tissue destructionR01EY024630 · NEI · GEORGIA STATE UNIVERSITY · PI DIX, RICHARD D · 2015 to 2019
$1.9MsnoRNA31 is critical for human cell-intrinsic immunity to HSV-1 in the central nervous systemR21AI151663 · NIAID · ROCKEFELLER UNIVERSITY · PI ZHANG, SHEN-YING · 2020 to 2021
$466kTranscriptional regulators of iris muscle cell developmentR21EY030215 · NEI · ICAHN SCHOOL OF MEDICINE AT MOUNT SINAI · PI BLENKINSOP, TIMOTHY A. · 2020 to 2021
$457kAIDS-related HSV-1 retinal necrosis: Innate immunity and virus spreadR21EY032215 · NEI · GEORGIA STATE UNIVERSITY · PI DIX, RICHARD D · 2021 to 2022
$429kRole of receptor-interacting serine/threonine-protein kinase 3 (RIPK3) in Herpes Simplex Virus (HSV) EncephalitisR21AI175590 · NIAID · LOUISIANA STATE UNIV HSC SHREVEPORT · PI GUO, HONGYAN · 2023 to 2024
$402kHoward Hughes Medical InstituteNCATS NIH HHS UL1 TR001866NCI NIH HHS P30 CA008748NEI NIH HHS R01 EY024630NEI NIH HHS R21 EY030215NEI NIH HHS R21 EY032215NIAID NIH HHS R01 AI020211NIAID NIH HHS R01 AI088364NIAID NIH HHS R21 AI151663NIAID NIH HHS R21 AI175590NINDS NIH HHS R01 NS072381
Abstract
Inborn errors of TLR3-dependent type I IFN immunity in cortical neurons underlie forebrain herpes simplex virus-1 (HSV-1) encephalitis (HSE) due to uncontrolled viral growth and subsequent cell death. We report an otherwise healthy patient with HSE who was compound heterozygous for nonsense (R422*) and frameshift (P493fs9*)
Indexed as
Encephalitis, Herpes SimplexHerpes SimplexHerpesvirus 1, HumanCell DeathHumansReceptor-Interacting Protein Serine-Threonine KinasesReceptors, Tumor Necrosis Factor, Type IToll-Like Receptor 3Receptor-Interacting Protein Serine-Threonine KinasesReceptors, Tumor Necrosis Factor, Type IRIPK3 protein, humanToll-Like Receptor 3
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.