Unravelling the association of partial T-cell immunodeficiency and immune dysregulation
Date
2008
Authors
Liston, Adrian
Enders, Anselm
Siggs, Owen
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Nature Publishing Group
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.
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Keywords
Keywords: adenosine deaminase; antinuclear antibody; autoantibody; cre recombinase; cytokine; cytokine receptor; DNA; ethylnitrosourea; growth hormone; immunoglobulin E; immunoglobulin G1; immunoglobulin G2a; immunoglobulin G2b; immunoglobulin M; interleukin 2 rece
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Source
Nature Reviews Immunology
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Journal article
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Restricted until
2037-12-31