Review

Nature Reviews Immunology 8, 545-558 (July 2008) | doi:10.1038/nri2336

Unravelling the association of partial T-cell immunodeficiency and immune dysregulation

Adrian Liston1,2, Anselm Enders1 & Owen M. Siggs1,3  About the authors

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Partial T-cell immunodeficiencies constitute a heterogeneous cluster of disorders characterized by an incomplete reduction in T-cell number or activity. The immune deficiency component of these diseases is less severe than that of the severe T-cell immunodeficiencies and therefore some ability to respond to infectious organisms is retained. Unlike severe T-cell immunodeficiencies, however, partial immunodeficiencies are commonly associated with hyper-immune dysregulation, including autoimmunity, inflammatory diseases and elevated IgE production. This causative association is counter-intuitive — immune deficiencies are caused by loss-of-function changes to the T-cell component, whereas the coincident autoimmune symptoms are the consequence of gain-of-function changes. This Review details the genetic basis of partial T -cell immunodeficiencies and draws on recent advances in mouse models to propose mechanisms by which a reduction in T-cell numbers or function may disturb the population-dependent balance between activation and tolerance.

Author affiliations

  1. John Curtin School of Medical Research, Australian National University, Canberra, ACT 0200, Australia.
  2. Department of Immunology, University of Washington, Seattle, Washington 98195, USA.
  3. Department of Genetics, The Scripps Research Institute, 10550 North Torrey Pines Road, La Jolla, California 92037, USA.

Correspondence to: Adrian Liston1,2 Email: liston@u.washington.edu

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