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T and B-cell signaling in activated PI3K delta syndrome: From immunodeficiency to autoimmunity.

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  • معلومة اضافية
    • المصدر:
      Publisher: Blackwell Country of Publication: England NLM ID: 7702118 Publication Model: Print Cited Medium: Internet ISSN: 1600-065X (Electronic) Linking ISSN: 01052896 NLM ISO Abbreviation: Immunol Rev Subsets: MEDLINE
    • بيانات النشر:
      Publication: <2002-> : Oxford : Blackwell
      Original Publication: Copenhagen, Munksgaard.
    • الموضوع:
      Signal Transduction*; B-Lymphocytes/*metabolism ; Class I Phosphatidylinositol 3-Kinases/*metabolism ; T-Lymphocytes/*metabolism; Animals ; Autoimmunity ; B-Lymphocytes/immunology ; Biomarkers ; Disease Susceptibility ; Energy Metabolism ; Humans ; Immunotherapy ; Neoplasms/etiology ; Neoplasms/metabolism ; Neoplasms/therapy ; Primary Immunodeficiency Diseases/etiology ; Primary Immunodeficiency Diseases/metabolism ; T-Lymphocytes/immunology
    • نبذة مختصرة :
      Phosphatidylinositol 3 kinases (PI3K) are a family of lipid kinases that are activated by a variety of cell-surface receptors, and regulate a wide range of downstream readouts affecting cellular metabolism, growth, survival, differentiation, adhesion, and migration. The importance of these lipid kinases in lymphocyte signaling has recently been highlighted by genetic analyses, including the recognition that both activating and inactivating mutations of the catalytic subunit of PI3Kδ, p110δ, lead to human primary immunodeficiencies. In this article, we discuss how studies on the human genetic disorder "Activated PI3K-delta syndrome" and mouse models of this disease (Pik3cd E1020K/+ mice) have provided fundamental insight into pathways regulated by PI3Kδ in T and B cells and their contribution to lymphocyte function and disease, including responses to commensal bacteria and the development of autoimmunity and tumors. We highlight critical roles of PI3Kδ in T follicular helper cells and the orchestration of the germinal center reaction, as well as in CD8 + T-cell function. We further  present data demonstrating the ability of the AKT-resistant FOXO1 AAA mutant to rescue IgG1 class switching defects in Pik3cd E1020K/+ B cells, as well as data supporting a role for PI3Kδ in promoting multiple T-helper effector cell lineages.
      (Published 2019. This article is a U.S. Government work and is in the public domain in the USA.)
    • Contributed Indexing:
      Keywords: APDS/PASLI; T and B lymphocytes; autoimmunity; immunodeficiency; phosphatidylinositol 3 kinase
    • الرقم المعرف:
      0 (Biomarkers)
      EC 2.7.1.137 (Class I Phosphatidylinositol 3-Kinases)
    • الموضوع:
      Date Created: 20190813 Date Completed: 20200224 Latest Revision: 20200224
    • الموضوع:
      20221213
    • الرقم المعرف:
      10.1111/imr.12790
    • الرقم المعرف:
      31402502