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Cytokines and immunodeficiency diseases.

W J Leonard1

  • 1Laboratory of Molecular Immunology, National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, Maryland 20892-1674, USA. wjl@helix.nih.gov

Nature Reviews. Immunology
|March 22, 2002
PubMed
Summary

Severe combined immunodeficiency disease (SCID) involves inherited immune system defects. Genetic studies reveal critical roles for cytokine signaling pathways in lymphoid development and function, with notable human-mouse differences.

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Area of Science:

  • Immunology
  • Genetics
  • Cell Biology

Background:

  • Severe combined immunodeficiency disease (SCID) encompasses severe inherited immunodeficiencies.
  • Many immunodeficiencies stem from defects in cytokine signaling pathways.
  • These defects impact lymphoid lineage development and cell signaling.

Purpose of the Study:

  • To elucidate the role of cytokine signaling in human immunodeficiencies.
  • To compare human genetic data with mouse models of cytokine signaling.
  • To understand the non-redundant functions of specific cytokines and signaling molecules.

Main Methods:

  • Analysis of human genetic defects in cytokine signaling pathways.
  • Phenotypic comparison of human SCID cases with analogous mouse-knockout models.
  • Investigating the impact of signaling defects on lymphoid development.

Main Results:

  • Human genetic studies highlight essential roles for cytokine signaling in immunity.
  • Cytokine pathway defects impair lymphoid development and cell function.
  • Unexpected differences observed in cytokine signaling between human and mouse models.

Conclusions:

  • Cytokine signaling pathways are crucial for normal immune system development and function.
  • Human genetic 'experiments' reveal non-redundant cytokine roles.
  • Mouse models provide insights but do not fully replicate human cytokine signaling complexities.

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