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

dc.contributor.authorListon, Adrian
dc.contributor.authorEnders, Anselm
dc.contributor.authorSiggs, Owen
dc.date.accessioned2015-12-08T22:11:16Z
dc.date.issued2008
dc.date.updated2015-12-08T07:39:27Z
dc.description.abstractPartial 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.
dc.identifier.issn1474-1733
dc.identifier.urihttp://hdl.handle.net/1885/29712
dc.publisherNature Publishing Group
dc.sourceNature Reviews Immunology
dc.subjectKeywords: 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
dc.titleUnravelling the association of partial T-cell immunodeficiency and immune dysregulation
dc.typeJournal article
local.bibliographicCitation.lastpage558
local.bibliographicCitation.startpage545
local.contributor.affiliationListon, Adrian, College of Medicine, Biology and Environment, ANU
local.contributor.affiliationEnders, Anselm, College of Medicine, Biology and Environment, ANU
local.contributor.affiliationSiggs, Owen, College of Medicine, Biology and Environment, ANU
local.contributor.authoremailu4265664@anu.edu.au
local.contributor.authoruidListon, Adrian, u3937101
local.contributor.authoruidEnders, Anselm, u4265664
local.contributor.authoruidSiggs, Owen, u4102606
local.description.embargo2037-12-31
local.description.notesImported from ARIES
local.identifier.absfor110799 - Immunology not elsewhere classified
local.identifier.ariespublicationu6800332xPUB67
local.identifier.citationvolume80
local.identifier.doi10.1038/nri2336
local.identifier.scopusID2-s2.0-46249103136
local.identifier.thomsonID000257089200015
local.identifier.uidSubmittedByu6800332
local.type.statusPublished Version

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