Impaired intrinsic immunity to HSV-1 in human iPSC-derived TLR3-deficient CNS cells

Fabien G. Lafaille, Itai M. Pessach, Shen-Ying Zhang (), Michael J. Ciancanelli, Melina Herman, Avinash Abhyankar, Shui-Wang Ying, Sotirios Keros, Peter A. Goldstein, Gustavo Mostoslavsky, Jose Ordovas-Montanes, Emmanuelle Jouanguy, Sabine Plancoulaine, Edmund Tu, Yechiel Elkabetz, Saleh Al-Muhsen, Marc Tardieu, Thorsten M. Schlaeger, George Q. Daley, Laurent Abel, Jean-Laurent Casanova (), Lorenz Studer and Luigi D. Notarangelo
Additional contact information
Fabien G. Lafaille: Center for Stem Cell Biology, Sloan-Kettering Institute for Cancer Research
Itai M. Pessach: Children's Hospital, Harvard Medical School
Shen-Ying Zhang: St. Giles Laboratory of Human Genetics of Infectious Diseases, The Rockefeller University
Michael J. Ciancanelli: St. Giles Laboratory of Human Genetics of Infectious Diseases, The Rockefeller University
Melina Herman: St. Giles Laboratory of Human Genetics of Infectious Diseases, The Rockefeller University
Avinash Abhyankar: St. Giles Laboratory of Human Genetics of Infectious Diseases, The Rockefeller University
Shui-Wang Ying: C.V. Starr Laboratory for Molecular Neuropharmacology, Weill Cornell Medical College
Sotirios Keros: Weill Cornell Medical College
Peter A. Goldstein: C.V. Starr Laboratory for Molecular Neuropharmacology, Weill Cornell Medical College
Gustavo Mostoslavsky: Section of Gastroenterology, Boston University School of Medicine
Jose Ordovas-Montanes: Children's Hospital, Harvard Medical School
Emmanuelle Jouanguy: St. Giles Laboratory of Human Genetics of Infectious Diseases, The Rockefeller University
Sabine Plancoulaine: Laboratory of Human Genetics of Infectious Diseases, Institut National de la Santé et de la Recherche Médicale, University Paris Descartes, Necker Medical School, U980, Paris 75015, France
Edmund Tu: Center for Stem Cell Biology, Sloan-Kettering Institute for Cancer Research
Yechiel Elkabetz: Laboratory for Pluripotent and Neural Stem Cell Biology, Sackler School of Medicine, Tel Aviv University, Tel Aviv 69978, Israel
Saleh Al-Muhsen: Prince Naif Center for Immunology Research, College of Medicine, King Saud University, Riyadh 11451, Saudi Arabia
Marc Tardieu: Assistance Publique-Hôpitaux de Paris, Bicêtre Hospital, Kremlin-Bicêtre, 94275, France
Thorsten M. Schlaeger: Children's Hospital and Dana-Farber Cancer Institute
George Q. Daley: Children's Hospital and Dana-Farber Cancer Institute
Laurent Abel: St. Giles Laboratory of Human Genetics of Infectious Diseases, The Rockefeller University
Jean-Laurent Casanova: St. Giles Laboratory of Human Genetics of Infectious Diseases, The Rockefeller University
Lorenz Studer: Center for Stem Cell Biology, Sloan-Kettering Institute for Cancer Research
Luigi D. Notarangelo: Children's Hospital, Harvard Medical School

Nature, 2012, vol. 491, issue 7426, 769-773

Abstract: Neurons and oligodendrocytes differentiated from induced pluripotent stem cells (iPSCs) of patients with inherited TLR3 and UNC-93B deficiencies are found to be more susceptible to infection by HSV-1 than control cells because they fail to induce a normal interferon response, whereas neural stem cells and astrocytes are not susceptible.

Date: 2012
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DOI: 10.1038/nature11583

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