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J-GLOBAL ID：201802284333330725   Reference number：18A0508563

Inborn Errors of RNA Lariat Metabolism in Humans with Brainstem Viral Infection

脳幹ウイルスに感染したヒトにおけるRNAラリアット先天性代謝異常【Powered by NICT】

Author (72)： Zhang Shen-Ying (St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY 10065, USA) ,  Zhang Shen-Ying (Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, Paris 75015, France) ,  Zhang Shen-Ying (Paris Descartes University, Imagine Institute, Paris 75015, France) ,  Clark Nathaniel E. (Department of Biochemistry and Structural Biology, University of Texas Health Science Center at San Antonio, San Antonio, TX 78229, USA) ,  Freije Catherine A. (Program in Immunogenomics, The Rockefeller University, New York, NY 10065, USA) ,  Pauwels Elodie (St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY 10065, USA) ,  Taggart Allison J. (Department of Molecular Biology, Cell Biology and Biochemistry, Brown University, Providence, RI 02912, USA) ,  Okada Satoshi (Department of Pediatrics, Hiroshima University Graduate School of Biomedical & Health Sciences, Hiroshima 734-8553, Japan) ,  Mandel Hanna (Metabolic Unit, Ruth Children’s Hospital, Rambam Health Care Campus, Haifa 31096, Israel) ,  Mandel Hanna (Rappaport Faculty of Medicine, Haifa 31096, Israel) ,  Garcia Paula (Child Developmental Center, Pediatric Hospital, Hospital and University Center of Coimbra, Coimbra 3000-602, Portugal) ,  Ciancanelli Michael J. (St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY 10065, USA) ,  Biran Anat (St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY 10065, USA) ,  Lafaille Fabien G. (St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY 10065, USA) ,  Tsumura Miyuki (Department of Pediatrics, Hiroshima University Graduate School of Biomedical & Health Sciences, Hiroshima 734-8553, Japan) ,  Cobat Aurelie (Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, Paris 75015, France) ,  Cobat Aurelie (Paris Descartes University, Imagine Institute, Paris 75015, France) ,  Luo Jingchuan (Department of Molecular Biology & Genetics, JHU School of Medicine, Baltimore, MD 21205, USA) ,  Luo Jingchuan (Institute for Systems Genetics, NYU Langone Health, New York 10016, NY, USA) ,  Volpi Stefano (Pediatric and Rheumatology Clinic, Center for Autoinflammatory Diseases and Immunodeficiencies, Istituto Giannina Gaslini and University of Genoa, Genoa 16100, Italy) ,  Zimmer Bastian (The Center for Stem Cell Biology, Sloan Kettering Institute for Cancer Research, New York, NY 10065, USA) ,  Sakata Sonoko (Department of Pediatrics, Hiroshima University Graduate School of Biomedical & Health Sciences, Hiroshima 734-8553, Japan) ,  Dinis Alexandra (Pediatric Intensive Care Unit, Pediatric Hospital, Hospital and University Center of Coimbra, Coimbra 3000-075, Portugal) ,  Ohara Osamu (Department of Technology Development, Kazusa DNA Research Institute, Chiba 292-0818, Japan) ,  Ohara Osamu (Laboratory for Integrative Genomics, RIKEN Center for Integrative Medical Sciences, Yokohama 230-0045, Japan) ,  Garcia Reino Eduardo J. (St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY 10065, USA) ,  Dobbs Kerry (Laboratory of Clinical Immunology and Microbiology, National Institute of Allergy and Infectious Diseases, NIH, Bethesda, MD 20892-1456, USA) ,  Hasek Mary (St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY 10065, USA) ,  Holloway Stephen P. (Department of Biochemistry and Structural Biology, University of Texas Health Science Center at San Antonio, San Antonio, TX 78229, USA) ,  McCammon Karen (Department of Biochemistry and Structural Biology, University of Texas Health Science Center at San Antonio, San Antonio, TX 78229, USA) ,  Hussong Stacy A. (Department of Cellular and Integrative Physiology and The Barshop Institute for Longevity and Aging Studies, University of Texas Health Science Center at San Antonio, TX 78229, USA) ,  Hussong Stacy A. (South Texas Veterans Health Care System, Department of Veterans Affairs, TX 78229, USA) ,  DeRosa Nicholas (Department of Cellular and Integrative Physiology and The Barshop Institute for Longevity and Aging Studies, University of Texas Health Science Center at San Antonio, TX 78229, USA) ,  Van Skike Candice E. (Department of Cellular and Integrative Physiology and The Barshop Institute for Longevity and Aging Studies, University of Texas Health Science Center at San Antonio, TX 78229, USA) ,  Katolik Adam (Department of Chemistry, McGill University, Montreal, QC H3A0B8, Canada) ,  Lorenzo Lazaro (Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, Paris 75015, France) ,  Lorenzo Lazaro (Paris Descartes University, Imagine Institute, Paris 75015, France) ,  Hyodo Maki (Department of Obstetrics and Gynecology, Hiroshima University Graduate School of Biomedical & Health Sciences, Hiroshima 734-8553, Japan) ,  Faria Emilia (Immuno-Allergy Department, Hospital and University of Coimbra, Coimbra 3000-075, Portugal) ,  Halwani Rabih (Immunology Research Laboratory, Department of Pediatrics, College of Medicine, King Saud University, Riyadh 11461, Saudi Arabia) ,  Fukuhara Rie (Department of Neonatology, Hiroshima Prefectural Hospital, Hiroshima 734-8115, Japan) ,  Smith Gregory A. (Department of Microbiology-Immunology, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA) ,  Galvan Veronica (Department of Cellular and Integrative Physiology and The Barshop Institute for Longevity and Aging Studies, University of Texas Health Science Center at San Antonio, TX 78229, USA) ,  Galvan Veronica (South Texas Veterans Health Care System, Department of Veterans Affairs, TX 78229, USA) ,  Damha Masad J. (Department of Chemistry, McGill University, Montreal, QC H3A0B8, Canada) ,  Al-Muhsen Saleh (Immunology Research Laboratory, Department of Pediatrics, College of Medicine, King Saud University, Riyadh 11461, Saudi Arabia) ,  Itan Yuval (St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY 10065, USA) ,  Itan Yuval (The Charles Bronfman Institute for Personalized Medicine, Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA) ,  Itan Yuval (Department of Genetics and Genomic Sciences, Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA) ,  Boeke Jef D. (Department of Molecular Biology & Genetics, JHU School of Medicine, Baltimore, MD 21205, USA) ,  Boeke Jef D. (Institute for Systems Genetics, NYU Langone Health, New York 10016, NY, USA) ,  Notarangelo Luigi D. (Laboratory of Clinical Immunology and Microbiology, National Institute of Allergy and Infectious Diseases, NIH, Bethesda, MD 20892-1456, USA) ,  Studer Lorenz (The Center for Stem Cell Biology, Sloan Kettering Institute for Cancer Research, New York, NY 10065, USA) ,  Kobayashi Masao (Department of Pediatrics, Hiroshima University Graduate School of Biomedical & Health Sciences, Hiroshima 734-8553, Japan) ,  Diogo Luisa (Pediatric Hospital of Coimbra, Coimbra 3000-075, Portugal) ,  Fairbrother William G. (Department of Molecular Biology, Cell Biology and Biochemistry, Brown University, Providence, RI 02912, USA) ,  Fairbrother William G. (Hassenfeld Child Health Innovation Institute, Brown University, Providence, RI 02912, USA) ,  Abel Laurent (St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY 10065, USA) ,  Abel Laurent (Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, Paris 75015, France) ,  Abel Laurent (Paris Descartes University, Imagine Institute, Paris 75015, France) ,  Rosenberg Brad R. (Program in Immunogenomics, The Rockefeller University, New York, NY 10065, USA) ,  Rosenberg Brad R. (Department of Microbiology, Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA) ,  Hart P. John (Department of Biochemistry and Structural Biology, University of Texas Health Science Center at San Antonio, San Antonio, TX 78229, USA) ,  Hart P. John (South Texas Veterans Health Care System, Department of Veterans Affairs, TX 78229, USA) ,  Hart P. John (X-ray Crystallography Core Laboratory, University of Texas Health Science Center at San Antonio, San Antonio, TX 78229, USA) ,  Etzioni Amos (Rappaport Faculty of Medicine, Haifa 31096, Israel) ,  Etzioni Amos (Immunology Unit, Ruth Children’s Hospital, Haifa 31096, Israel) ,  Casanova Jean-Laurent (St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY 10065, USA) ,  Casanova Jean-Laurent (Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, Paris 75015, France) ,  Casanova Jean-Laurent (Paris Descartes University, Imagine Institute, Paris 75015, France) ,  Casanova Jean-Laurent (Howard Hughes Medical Institute, New York, NY 10065, USA) ,  Casanova Jean-Laurent (Pediatric Immunology-Hematology Unit, Necker Hospital for Sick Children, Paris 75015, France)
Material： Cell (Cambridge, Mass.)  (Cell (Camb. Mass.))
Volume： 172  Issue： 5  Page： 952-965.e18  Publication year： 2018 
JST Material Number： A0707B  ISSN： 0092-8674  Document type： Article
Article type： 原著論文  Country of issue： United States (USA)  Language： ENGLISH (EN)

Abstract/Point： Viruses that are typically ben...

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Thesaurus term： fibroblast ,  influenza virus ,  child ,  sensitivity ,  autosome ,  wild type ,  *virus ,  variation(biology) ,  allele ,  *RNA ,  *human(primates) ,  *inborn errors of metabolism ,  *brain stem ,  patient ,  viral infection

Author keywords (4)： DBR1 ,  RNA lariat debranching ,  viral encephalitis ,  brainstem

JST classification (2)： Cell physiology in general  (EF02010S) ,  Biological function  (EB03040U)

Terms in the title (6)： 脳幹 ,  ウイルス ,  感染 ,  ヒト ,  RNA ,  先天性代謝異常