ArticleNature medicine2019
Human SNORA31 variations impair cortical neuron-intrinsic immunity to HSV-1 and underlie herpes simplex encephalitis.
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Article in Nature medicine, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 79 papers.
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79citing papers in PubMed
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Who cites it
79 citing papers in PubMed, 112 citations in OpenAlex.
- In vivo CRISPR gene editing in patients with herpetic stromal keratitis.Molecular therapy : the journal of the American Society of Gene Therapy · 2023Trial
- Host Genetic Susceptibility and Antiviral Immunity Shape the Pathogenesis and Outcomes of Herpes Simplex Encephalitis.Biology · 2026Review
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- The cell-mediated adaptive immune response to herpes simplex virus type 1 encephalitis: mechanisms and clinical implications.Frontiers in immunology · 2026Review
- From chromatin to proteostasis: multilevel gene regulation in Mendelian diseases.Frontiers in epigenetics and epigenomics · 2026Review
- Human inborn errors of type I interferon-independent intrinsic immunity in nonleukocytic cells.Current opinion in immunology · 2025Review
- Small Nucleolar RNAs: Biological Functions and Diseases.MedComm · 2025Review
- Differentially expressed ncRNAs as key regulators in infection of human bronchial epithelial cells by the SARS-CoV-2 Delta variant.Molecular therapy. Nucleic acids · 2025Article
- Involvement of virus infections and antiviral agents in schizophrenia.Psychological medicine · 2025Review
- The monogenic landscape of human infectious diseases.The Journal of allergy and clinical immunology · 2025Review
- The immunogenetic basis of severe herpes simplex infections in neonates and children: a review.Pediatric research · 2025Review
- Ex vivo study of neuroinvasive and neurotropic viruses: what is current and what is next.FEMS microbiology reviews · 2025Review
- Human DBR1 deficiency impairs stress granule-dependent PKR antiviral immunity.The Journal of experimental medicine · 2025Article
- Defects in Innate and Intrinsic Immunity in Morocco: A Retrospective Analysis of the Genetic Landscape and Clinical Correlations.Pathogens & immunity · 2025Article
- Proteomic profiling of neonatal herpes simplex virus infection on dried blood spots.Communications medicine · 2024Article
- CNS disease associated with enhanced type I interferon signalling.The Lancet. Neurology · 2024Review
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- Orchestration of antiviral responses within the infected central nervous system.Cellular & molecular immunology · 2024Review
- From a focal skin issue to a systemic disease: the multifaceted nature of cold sores, novel findings.Virusdisease · 2024Article
19 more citing papers are in PubMed but not listed here.
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Authors and funding
43 authors at 18 institutions in 9 countries.
Fabien G LafailleSt. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY, USA.
Oliver HarschnitzThe Center for Stem Cell Biology, Sloan-Kettering Institute for Cancer Research, New York, NY, USA.ORCID http://orcid.org/0000-0001-6496-881X
Yoon Seung LeeSt. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY, USA.
Peng ZhangSt. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY, USA.
Mary L HasekSt. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY, USA.
Gaspard KernerLaboratory of Human Genetics of Infectious Diseases, Necker Branch, Necker Hospital for Sick Children, Paris, France.
Yuval ItanSt. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY, USA.
Osefame EwaleifohDepartment of Microbiology-Immunology, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
Franck RapaportSt. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY, USA.
Thomas M CarlileBiogen, Cambridge, MA, USA.
Madalina E Carter-TimofteDepartment of Infectious Diseases, Aarhus University Hospital, Aarhus, Denmark.
Dominik PaquetLaboratory of Brain Development and Repair, The Rockefeller University, New York, NY, USA.
Kerry DobbsLaboratory of Clinical Immunology and Microbiology, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA.ORCID http://orcid.org/0000-0002-3432-3137
Bastian ZimmerThe Center for Stem Cell Biology, Sloan-Kettering Institute for Cancer Research, New York, NY, USA.
Daxing GaoSt. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY, USA.
Maria F Rojas-DuranDepartment of Molecular Biophysics and Biochemistry, Yale University, New Haven, CT, USA.
Dylan KwartLaboratory of Brain Development and Repair, The Rockefeller University, New York, NY, USA.
Vimel RattinaLaboratory of Human Genetics of Infectious Diseases, Necker Branch, Necker Hospital for Sick Children, Paris, France.ORCID http://orcid.org/0000-0002-0801-0102
Michael J CiancanelliSt. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY, USA.
Jessica L McAlpineThe 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, Necker Hospital for Sick Children, Paris, France.
Soraya BoucheritLaboratory of Human Genetics of Infectious Diseases, Necker Branch, Necker Hospital for Sick Children, Paris, France.ORCID http://orcid.org/0000-0002-8819-7594
Flore RozenbergVirology Department, Paris Descartes University, Sorbonne Paris Cité University, Welfare Services Paris Hospital, Hospital Group Paris Center University, Paris, France.
Rabih HalwaniSharjah Institute for Medical Research (SIMR), Department of Clinical Sciences, College of Medicine, University of Sharjah, Sharjah, United Arab Emirates.
Benoit HenryDepartment of Infectious Diseases, Pitié-Salpêtrière Hospital, Paris, France.
Naima AmenzouiClinical Immunology Unit, Children's Ibn Rushd Hospital and Clinical Immunology Laboratory, Inflammation and Allergy LICIA, Faculty of Medicine and Pharmacy, Hassan Ii University, Casablanca, Morocco.
Zobaida AlsumImmunology Research Laboratory, Department of Pediatrics, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
Laura MarquesPediatric Department, Infectious Diseases and Immunodeficiencies Unit, Porto Hospital Center, Porto, Portugal.
Joseph A ChurchDepartment of Pediatrics, Children's Hospital Los Angeles and Keck School of Medicine, University of Southern California, Los Angeles, CA, USA.
Saleh Al-MuhsenPrince Naif Center for Immunology Research, King Saud University, Riyadh, Saudi Arabia.
Marc TardieuSouth Paris University Hospital, Paris Hospital Welfare Services, Department of Pediatric Neurology, Paris, France.
Ahmed Aziz BousfihaClinical Immunology Unit, Children's Ibn Rushd Hospital and Clinical Immunology Laboratory, Inflammation and Allergy LICIA, Faculty of Medicine and Pharmacy, Hassan Ii University, Casablanca, Morocco.
Søren R PaludanDepartment of Biomedicine, Aarhus University, Aarhus, Denmark.ORCID http://orcid.org/0000-0001-9180-4060
Trine Hyrup MogensenDepartment of Infectious Diseases, Aarhus University Hospital, Aarhus, Denmark.
Lluis Quintana-MurciUnit of Human Evolutionary Genetics, CNRS UMR2000, Institut Pasteur, Paris, France.
Marc Tessier-LavigneBiogen, Cambridge, MA, USA.
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, National Institutes of Health, Bethesda, MD, USA.ORCID http://orcid.org/0000-0002-8335-0262
Lorenz StuderThe Center for Stem Cell Biology, Sloan-Kettering Institute for Cancer Research, New York, NY, USA.ORCID http://orcid.org/0000-0003-0741-7987
Wendy GilbertDepartment of Molecular Biophysics and Biochemistry, Yale University, New Haven, CT, USA.
Laurent AbelSt. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY, USA.
Jean-Laurent CasanovaSt. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY, USA. casanova@rockefeller.edu.ORCID http://orcid.org/0000-0002-7782-4169
Shen-Ying ZhangSt. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY, USA. shzh289@rockefeller.edu.ORCID http://orcid.org/0000-0002-9449-3672
Rockefeller University · USUniversité Paris Cité · FRKettering University · USAarhus University · DKBiogen (United States) · USKing Saud University · SANational Institutes of Health · USNorthwestern University · USUniversity of Hassan II Casablanca · MAYale University · USCentre National de la Recherche Scientifique · FRCUF Porto Hospital · PTDélégation Paris 5 · FRHoward Hughes Medical Institute · USLMU Klinikum · DESorbonne Université · FRUniversity of Sharjah · AEUniversity of Southern California · US
Funding
X-RAY CRYSTALLOGRAPHYP30CA008748 · NCI · SLOAN-KETTERING INSTITUTE FOR CANCER RES · PI Nicholas D Socci · 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
$40.6MTransforming Translational Science and Education to Benefit Human HealthUL1TR000043 · NCATS · ROCKEFELLER UNIVERSITY · PI COLLER, BARRY · 2012 to 2015
$26.8MStudy 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
$8.7MRegulation and Function of snoRNA GenesR01GM101316 · NIGMS · YALE UNIVERSITY · PI Wendy Victoria Gilbert · 2014 to 2026
$3.9MsnoRNA31 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
$466kNCATS NIH HHS UL1 TR000043NCATS NIH HHS UL1 TR001866NCI NIH HHS P30 CA008748NIAID NIH HHS R01 AI088364NIAID NIH HHS R21 AI151663NIGMS NIH HHS R01 GM101316NINDS NIH HHS R01 NS072381
Abstract
Herpes simplex virus-1 (HSV-1) encephalitis (HSE) is typically sporadic. Inborn errors of TLR3- and DBR1-mediated central nervous system cell-intrinsic immunity can account for forebrain and brainstem HSE, respectively. We report five unrelated patients with forebrain HSE, each heterozygous for one of four rare variants of SNORA31, encoding a small nucleolar RNA of the H/ACA class that are predicted to direct the isomerization of uridine residues to pseudouridine in small nuclear RNA and ribosomal RNA. We show that CRISPR/Cas9-introduced bi- and monoallelic SNORA31 deletions render human pluripotent stem cell (hPSC)-derived cortical neurons susceptible to HSV-1. Accordingly, SNORA31-mutated patient hPSC-derived cortical neurons are susceptible to HSV-1, like those from TLR3- or STAT1-deficient patients. Exogenous interferon (IFN)-β renders SNORA31- and TLR3- but not STAT1-mutated neurons resistant to HSV-1. Finally, transcriptome analysis of SNORA31-mutated neurons revealed normal responses to TLR3 and IFN-α/β stimulation but abnormal responses to HSV-1. Human SNORA31 thus controls central nervous system neuron-intrinsic immunity to HSV-1 by a distinctive mechanism.
Indexed as
AdultCentral Nervous SystemChild, PreschoolEncephalitis, Herpes SimplexFemaleGenetic Predisposition to DiseaseHerpesvirus 1, HumanHumansImmunityInfantMaleMetagenomeMiddle AgedNeuronsRNA, Small NucleolarRNA, Small Nucleolar
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