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Autoimmune Lymphoproliferative Syndrome: Types I, II and Beyond

  • Chapter
Mechanisms of Lymphocyte Activation and Immune Regulation VIII

Part of the book series: Advances in Experimental Medicine and Biology ((AEMB,volume 490))

Abstract

An essential element in the maintenance of homeostasis of the immune system is the capacity for apoptosis, or programmed cell death. Apoptosis limits the accumulation of lymphocytes, and minimizes reactions against self-antigen that can lead to autoimmunity1,2. Lymphocyte apoptosis occurs in at least two major forms: antigen receptor engagement and lymphokine withdrawal. These forms of death are controlled in a negative feedback mechanism termed propriocidal regulation1,3. Antigen-mediated death of lymphocytes is regulated by Fas (CD95/APO-1), tumor necrosis factor receptor (TNFR), and related molecules4-6. Passive apoptosis via lymphokine withdrawal may result from the cytoplasmic activation of caspases that is closely regulated by the mitochondria and the members of the Bcl-2 family of proteins. Apoptosis of other immune cells such as dendritic cells may also contribute to immune homeostasis7.

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Author information

Authors and Affiliations

  1. Laboratory of Immunology, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland, 20892, USA

    Hyung J. Chun & Michael J. Lenardo

  2. Howard Hughes Medical Institute—National Institutes of Health Research Scholars Program, USA

    Hyung J. Chun

Authors
  1. Hyung J. Chun
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  2. Michael J. Lenardo
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Editors and Affiliations

  1. University of California, Irvine, Irvine, California, USA

    Sudhir Gupta

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© 2001 Springer Science+Business Media New York

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Chun, H.J., Lenardo, M.J. (2001). Autoimmune Lymphoproliferative Syndrome: Types I, II and Beyond. In: Gupta, S. (eds) Mechanisms of Lymphocyte Activation and Immune Regulation VIII. Advances in Experimental Medicine and Biology, vol 490. Springer, Boston, MA. https://doi.org/10.1007/978-1-4615-1243-1_6

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  • DOI: https://doi.org/10.1007/978-1-4615-1243-1_6

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4613-5458-1

  • Online ISBN: 978-1-4615-1243-1

  • eBook Packages: Springer Book Archive

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