Unravelling the association of partial T-cell immunodeficiency and immune dysregulation
dc.contributor.author | Liston, Adrian | |
dc.contributor.author | Enders, Anselm | |
dc.contributor.author | Siggs, Owen | |
dc.date.accessioned | 2015-12-08T22:11:16Z | |
dc.date.issued | 2008 | |
dc.date.updated | 2015-12-08T07:39:27Z | |
dc.description.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. | |
dc.identifier.issn | 1474-1733 | |
dc.identifier.uri | http://hdl.handle.net/1885/29712 | |
dc.publisher | Nature Publishing Group | |
dc.source | Nature Reviews Immunology | |
dc.subject | 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 | |
dc.title | Unravelling the association of partial T-cell immunodeficiency and immune dysregulation | |
dc.type | Journal article | |
local.bibliographicCitation.lastpage | 558 | |
local.bibliographicCitation.startpage | 545 | |
local.contributor.affiliation | Liston, Adrian, College of Medicine, Biology and Environment, ANU | |
local.contributor.affiliation | Enders, Anselm, College of Medicine, Biology and Environment, ANU | |
local.contributor.affiliation | Siggs, Owen, College of Medicine, Biology and Environment, ANU | |
local.contributor.authoremail | u4265664@anu.edu.au | |
local.contributor.authoruid | Liston, Adrian, u3937101 | |
local.contributor.authoruid | Enders, Anselm, u4265664 | |
local.contributor.authoruid | Siggs, Owen, u4102606 | |
local.description.embargo | 2037-12-31 | |
local.description.notes | Imported from ARIES | |
local.identifier.absfor | 110799 - Immunology not elsewhere classified | |
local.identifier.ariespublication | u6800332xPUB67 | |
local.identifier.citationvolume | 80 | |
local.identifier.doi | 10.1038/nri2336 | |
local.identifier.scopusID | 2-s2.0-46249103136 | |
local.identifier.thomsonID | 000257089200015 | |
local.identifier.uidSubmittedBy | u6800332 | |
local.type.status | Published Version |
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