Journal
NATURE REVIEWS IMMUNOLOGY
Volume 8, Issue 7, Pages 545-558Publisher
NATURE PUBLISHING GROUP
DOI: 10.1038/nri2336
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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.
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