Combined immunodeficiency with autoimmunity caused by a homozygous missense mutation in inhibitor of nuclear factor 𝛋B kinase alpha (IKKα)

  • Wayne Bainter
    Division of Immunology, Boston Children’s Hospital, Harvard Medical School, Boston, MA, USA.
  • Vassilios Lougaris
    Pediatrics Clinic, Institute for Molecular Medicine A. Nocivelli, Department of Clinical and Experimental Sciences, University of Brescia, ASST-Spedali Civili of Brescia, Brescia, Italy.
  • Jacqueline G. Wallace
    Division of Immunology, Boston Children’s Hospital, Harvard Medical School, Boston, MA, USA.
  • Yousef Badran
    Division of Immunology, Boston Children’s Hospital, Harvard Medical School, Boston, MA, USA.
  • Rodrigo Hoyos-Bachiloglu
    Division of Immunology, Boston Children’s Hospital, Harvard Medical School, Boston, MA, USA.
  • Zachary Peters
    Division of Immunology, Boston Children’s Hospital, Harvard Medical School, Boston, MA, USA.
  • Hazel Wilkie
    Division of Immunology, Boston Children’s Hospital, Harvard Medical School, Boston, MA, USA.
  • Mrinmoy Das
    Division of Immunology, Boston Children’s Hospital, Harvard Medical School, Boston, MA, USA.
  • Erin Janssen
    Division of Immunology, Boston Children’s Hospital, Harvard Medical School, Boston, MA, USA.
  • Abdallah Beano
    Division of Immunology, Boston Children’s Hospital, Harvard Medical School, Boston, MA, USA.
  • Khaoula Ben Farhat
    Division of Immunology, Boston Children’s Hospital, Harvard Medical School, Boston, MA, USA.
  • Christy Kam
    Division of Immunology, Boston Children’s Hospital, Harvard Medical School, Boston, MA, USA.
  • Luisa Bercich
    Department of Pathology, University of Brescia, ASST Spedali Civili of Brescia, Brescia, Italy.
  • Paolo Incardona
    Department of Pathology, University of Brescia, ASST Spedali Civili of Brescia, Brescia, Italy.
  • Vincenzo Villanacci
    Department of Pathology, University of Brescia, ASST Spedali Civili of Brescia, Brescia, Italy.
  • Maria Pia Bondioni
    Department of Pediatric Radiology, University of Brescia, ASST Spedali Civili of Brescia, Brescia, Italy.
  • Antonella Meini
    Pediatrics Clinic, Institute for Molecular Medicine A. Nocivelli, Department of Clinical and Experimental Sciences, University of Brescia, ASST-Spedali Civili of Brescia, Brescia, Italy.
  • Manuela Baronio
    Pediatrics Clinic, Institute for Molecular Medicine A. Nocivelli, Department of Clinical and Experimental Sciences, University of Brescia, ASST-Spedali Civili of Brescia, Brescia, Italy.
  • Phammela Abarzua
    Department of Dermatology, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA.
  • Silvia Parolini
    Pediatrics Clinic, Institute for Molecular Medicine A. Nocivelli, Department of Clinical and Experimental Sciences, University of Brescia, ASST-Spedali Civili of Brescia, Brescia, Italy.
  • Giovanna Tabellini
    Pediatrics Clinic, Institute for Molecular Medicine A. Nocivelli, Department of Clinical and Experimental Sciences, University of Brescia, ASST-Spedali Civili of Brescia, Brescia, Italy.
  • Stefano Maio
    Department of Paediatrics, the Weatherall Institute of Molecular Medicine, University of Oxford, Oxford, UK.
  • Birgitta Schmidt
    Department of Pathology, Boston Children’s Hospital, Harvard Medical School, Boston, MA, USA.
  • Jeffrey D. Goldsmith
    Department of Pathology, Boston Children’s Hospital, Harvard Medical School, Boston, MA, USA.
  • George Murphy
    Department of Dermatology, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA.
  • Georg Hollander
    Department of Paediatrics, the Weatherall Institute of Molecular Medicine, University of Oxford, Oxford, UK.
  • Alessandro Plebani
    Pediatrics Clinic, Institute for Molecular Medicine A. Nocivelli, Department of Clinical and Experimental Sciences, University of Brescia, ASST-Spedali Civili of Brescia, Brescia, Italy.
  • Janet Chou
    Division of Immunology, Boston Children’s Hospital, Harvard Medical School, Boston, MA, USA.
  • Raif S. Geha
    Division of Immunology, Boston Children’s Hospital, Harvard Medical School, Boston, MA, USA.

説明

<jats:p>A mutation in human IKKα compromises noncanonical NF-κB signaling resulting in primary immunodeficiency associated with autoimmunity.</jats:p>

収録刊行物

  • Science Immunology

    Science Immunology 6 (63), eabf6723-, 2021-09-24

    American Association for the Advancement of Science (AAAS)

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