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Nat Rev Immunol. 2008 Jul;8(7):545-58. doi: 10.1038/nri2336.

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

Author information

1
John Curtin School of Medical Research, Australian National University, Canberra, ACT 0200, Australia. liston@u.washington.edu

Abstract

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.

PMID:
18551129
DOI:
10.1038/nri2336
[Indexed for MEDLINE]

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