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Title: Unravelling the association of partial T-cell immunodeficiency and immune dysregulation
Authors: Liston, Adrian ×
Enders, Anselm
Siggs, Owen M #
Issue Date: Jul-2008
Publisher: Nature Pub. Group
Series Title: Nature Reviews. Immunology vol:8 issue:7 pages:545-558
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.
ISSN: 1474-1733
Publication status: published
KU Leuven publication type: IT
Appears in Collections:Department of Experimental Medicine (-)
Laboratory of Genetics of Autoimmunity
× corresponding author
# (joint) last author

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