Advertisement

Molecular and Cellular Basis of Immunity and Immunological Diseases

  • Kevin Elias
  • Richard Siegel
  • John J. O’Shea

Abstract

The vertebrate immune system protects the host from a wide variety of pathogens, including bacteria, viruses, fungi, and parasites. However, this remarkable versatility comes at the cost of autoimmune and autoinflammatory diseases that affect approximately 1 in 30 individuals. This chapter briefly reviews the cardinal molecular and cellular features of the immune system, including the molecular basis of recognition and response to pathogens, mechanisms of tolerance, and immunological memory. Knowledge of the immune system is critical to understanding rheumatologic disease. While the detailed pathophysiology of major autoimmune diseases remains elusive, genetic immune disorders and the effects of new biologic targeted treatments provide insights into many of the mechanisms of immunological disease.

Keywords

Natural Killer Cell Familial Mediterranean Fever Antigen Receptor Ataxia Telangiectasia Mutate Innate Immune Cell 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

References

  1. 1.
    Creagh EM, O’Neill LA. TLRs, NLRs, and RLRs: a trinity of pathogen sensors that co-operate in innate immunity. Trends Immunol 2006;27:352–357.CrossRefPubMedGoogle Scholar
  2. 2.
    Akira S, Uematsu S, Takeuchi O. Pathogen recognition and innate immunity. Cell 2006;124:783–801.CrossRefPubMedGoogle Scholar
  3. 3.
    Ogura Y, Sutterwala FS, Flavell RA. The inflammasome: first line of the immune response to cell stress. Cell 2006;126:659–662.CrossRefPubMedGoogle Scholar
  4. 4.
    Stojanov S, Kastner DL. Familial autoinflammatory diseases: genetics, pathogenesis and treatment. Curr Opin Rheumatol 2005;17:586–599.CrossRefPubMedGoogle Scholar
  5. 5.
    Steinman RM, Bonifaz L, Fujii S, et al. The innate functions of dendritic cells in peripheral lymphoid tissues. Adv Exp Med Biol 2005;560:83–97.CrossRefPubMedGoogle Scholar
  6. 6.
    Stetson DB, Medzhitov R. Type I interferons in host defense. Immunity 2006;25:373–381.CrossRefPubMedGoogle Scholar
  7. 7.
    Brigl M, Bry L, Kent SC, Gumperz JE, Brenner MB. Mechanism of CD1d-restricted natural killer T cell activation during microbial infection. Nat Immunol 2003;4:1230–1237.CrossRefPubMedGoogle Scholar
  8. 8.
    Prussin C, Metcalfe DD. IgE, mast cells, basophils, and eosinophils. Allergy Clin Immunol 2006;117(Suppl):S450–S456.CrossRefGoogle Scholar
  9. 9.
    Wen L, Atkinson JP, Giclas PC. Clinical and laboratory evaluation of complement deficiency. J Allergy Clin Immunol 2004;113:585–593.CrossRefPubMedGoogle Scholar
  10. 10.
    Kovanen PE, Leonard WJ. Cytokines and immunodeficiency diseases: critical roles of the gamma(c)-dependent cytokines interleukins 2, 4, 7, 9, 15, and 21, and their signaling pathways. Immunol Rev 2004;202:67–83.CrossRefPubMedGoogle Scholar
  11. 11.
    Davis SJ, van der Merwe PA. The kinetic-segregation model: TCR triggering and beyond. Nat Immunol 2006;7:803–809.CrossRefPubMedGoogle Scholar
  12. 12.
    O’Shea JJ, Husa M, Li D, et al. Jak3 and the pathogenesis of severe combined immunodeficiency. Mol Immunol 2004;41:727–737.CrossRefPubMedGoogle Scholar
  13. 13.
    Chikuma S, Bluestone JA. CTLA-4 and tolerance: the biochemical point of view. Immunol Res 2003;28:241–253.CrossRefPubMedGoogle Scholar
  14. 14.
    Villasenor J, Benoist C, Mathis D. AIRE and APECED: molecular insights into an autoimmune disease. Immunol Rev 2005;204:156–164.CrossRefPubMedGoogle Scholar
  15. 15.
    Laky K, Fowlkes BJ. Receptor signals and nuclear events in CD4 and CD8 T cell lineage commitment. Curr Opin Immunol 2005;17:116–121.CrossRefPubMedGoogle Scholar
  16. 16.
    Weaver CT, Harrington LE, Mangan PR, Gavrieli M, Murphy KM. Th17: an effector CD4 T cell lineage with regulatory T cell ties. Immunity 2006;24:677–688.CrossRefPubMedGoogle Scholar
  17. 17.
    Kronenberg M, Rudensky A. Regulation of immunity by self-reactive T cells. Nature 2005;435:598–604.CrossRefPubMedGoogle Scholar
  18. 18.
    Edwards JC, Cambridge G. B-cell targeting in rheuma-toid arthritis and other autoimmune diseases. Nat Rev Immunol 2006;6:394–403.CrossRefPubMedGoogle Scholar
  19. 19.
    Kalia V, Sarkar S, Gourley TS, Rouse BT, Ahmed R. Differentiation of memory B and T cells. Curr Opin Immunol 2006;18:255–264.CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2008

Authors and Affiliations

  • Kevin Elias
    • 1
  • Richard Siegel
    • 2
  • John J. O’Shea
    • 3
  1. 1.Lymphocyte Cell Biology SectionNational Institute of Arthritis, Musculoskeletal, and Skin DiseasesBethesdaUSA
  2. 2.Immunoregulation Group, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of HealthBethesdaUSA
  3. 3.Lymphocyte Cell Biology SectionNational Institutes of HealthBethesdaUSA

Personalised recommendations