Folicullar bronchiolitis as phenotype associated with CD25 deficiency

Autores
Bezrodnik, Liliana; Caldirola, M. S; Seminario, A. G.; Moreira, I.; Gaillard, M. I.
Año de publicación
2014
Idioma
inglés
Tipo de recurso
artículo
Estado
versión publicada
Descripción
Regulatory T cells (CD4+CD25+FOXp3+) are subsets of T cells involved in the maintenance of peripheral self tolerance by actively suppressing the activation and expansion of autoreactive T cells.1 Signaling through the interleukin-2 receptor (IL-2R) contributes to T-cell tolerance by controlling three important aspects of Treg biology. CD25 is the α-chain of the IL-2R that, in concert with the β-chain and γ-chain, constitutes the complete IL-2R. 2 CD25 only contributes to IL-2 binding affinity but not to the recruitment of signaling molecules. However, its importance in the development of a normal immune response is emphasized by the finding that a truncation mutant of CD25 results in an immunodeficiency in humans characterized by an increased susceptibility to viral, bacterial and fungal infections. 3 In 1997, Sharfe et al. described an infant with severe bacterial, viral, and fungal infections. Counts of autologous T lymphocytes were moderately low, T cells displayed a weak proliferative response to mitogens in vitro, and the patient displayed no rejection of an allogeneic skin graft. However, unlike children with SCID, the patient besides not having circulating T cells, also developed peripheral lymphocytic proliferation and autoimmune primary biliary cirrhosis. 4 We present the first female argentine patient with mutation in CD25 associated with chronic and severe inflammatory lung disease (Follicular bronchiolitis with lymphocyte hyperplasia), eczema and infections. She has no expression of CD25 on CD4+ T cells and extremely low amount of Tregs.
Fil: Bezrodnik, Liliana. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo Gutierrez"; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina
Fil: Caldirola, M. S. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo Gutierrez"; Argentina
Fil: Seminario, A. G.. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo Gutierrez"; Argentina
Fil: Moreira, I.. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo Gutierrez"; Argentina
Fil: Gaillard, M. I.. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo Gutierrez"; Argentina
Materia
Cd25 Deficiency
Foxp3
Follicular Brochiolitis
Ipex-Like
Nivel de accesibilidad
acceso abierto
Condiciones de uso
https://creativecommons.org/licenses/by-nc-sa/2.5/ar/
Repositorio
CONICET Digital (CONICET)
Institución
Consejo Nacional de Investigaciones Científicas y Técnicas
OAI Identificador
oai:ri.conicet.gov.ar:11336/3694
Acceder
id CONICETDig_5d8c83b976795fec40e302e496efc760
oai_identifier_str oai:ri.conicet.gov.ar:11336/3694
network_acronym_str CONICETDig
repository_id_str 3498
network_name_str CONICET Digital (CONICET)
spelling Folicullar bronchiolitis as phenotype associated with CD25 deficiencyBezrodnik, LilianaCaldirola, M. SSeminario, A. G.Moreira, I.Gaillard, M. I.Cd25 DeficiencyFoxp3Follicular BrochiolitisIpex-Likehttps://purl.org/becyt/ford/3.2https://purl.org/becyt/ford/3Regulatory T cells (CD4+CD25+FOXp3+) are subsets of T cells involved in the maintenance of peripheral self tolerance by actively suppressing the activation and expansion of autoreactive T cells.1 Signaling through the interleukin-2 receptor (IL-2R) contributes to T-cell tolerance by controlling three important aspects of Treg biology. CD25 is the α-chain of the IL-2R that, in concert with the β-chain and γ-chain, constitutes the complete IL-2R. 2 CD25 only contributes to IL-2 binding affinity but not to the recruitment of signaling molecules. However, its importance in the development of a normal immune response is emphasized by the finding that a truncation mutant of CD25 results in an immunodeficiency in humans characterized by an increased susceptibility to viral, bacterial and fungal infections. 3 In 1997, Sharfe et al. described an infant with severe bacterial, viral, and fungal infections. Counts of autologous T lymphocytes were moderately low, T cells displayed a weak proliferative response to mitogens in vitro, and the patient displayed no rejection of an allogeneic skin graft. However, unlike children with SCID, the patient besides not having circulating T cells, also developed peripheral lymphocytic proliferation and autoimmune primary biliary cirrhosis. 4 We present the first female argentine patient with mutation in CD25 associated with chronic and severe inflammatory lung disease (Follicular bronchiolitis with lymphocyte hyperplasia), eczema and infections. She has no expression of CD25 on CD4+ T cells and extremely low amount of Tregs.Fil: Bezrodnik, Liliana. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo Gutierrez"; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; ArgentinaFil: Caldirola, M. S. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo Gutierrez"; ArgentinaFil: Seminario, A. G.. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo Gutierrez"; ArgentinaFil: Moreira, I.. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo Gutierrez"; ArgentinaFil: Gaillard, M. I.. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo Gutierrez"; ArgentinaWiley2014-01-03info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionhttp://purl.org/coar/resource_type/c_6501info:ar-repo/semantics/articuloapplication/pdfapplication/pdfhttp://hdl.handle.net/11336/3694Bezrodnik, Liliana; Caldirola, M. S; Seminario, A. G.; Moreira, I.; Gaillard, M. I.; Folicullar bronchiolitis as phenotype associated with CD25 deficiency; Wiley; Clinical and Experimental Immunology; 175; 2; 3-1-2014; 227-2340009-9104enginfo:eu-repo/semantics/altIdentifier/url/http://onlinelibrary.wiley.com/doi/10.1111/cei.12214/abstractinfo:eu-repo/semantics/altIdentifier/doi/DOI:10.1111/cei.12214info:eu-repo/semantics/altIdentifier/issn/0009-9104info:eu-repo/semantics/altIdentifier/url/http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3892414/info:eu-repo/semantics/openAccesshttps://creativecommons.org/licenses/by-nc-sa/2.5/ar/reponame:CONICET Digital (CONICET)instname:Consejo Nacional de Investigaciones Científicas y Técnicas2025-09-03T09:52:11Zoai:ri.conicet.gov.ar:11336/3694instacron:CONICETInstitucionalhttp://ri.conicet.gov.ar/Organismo científico-tecnológicoNo correspondehttp://ri.conicet.gov.ar/oai/requestdasensio@conicet.gov.ar; lcarlino@conicet.gov.arArgentinaNo correspondeNo correspondeNo correspondeopendoar:34982025-09-03 09:52:11.472CONICET Digital (CONICET) - Consejo Nacional de Investigaciones Científicas y Técnicasfalse
dc.title.none.fl_str_mv Folicullar bronchiolitis as phenotype associated with CD25 deficiency
title Folicullar bronchiolitis as phenotype associated with CD25 deficiency
spellingShingle Folicullar bronchiolitis as phenotype associated with CD25 deficiency
Bezrodnik, Liliana
Cd25 Deficiency
Foxp3
Follicular Brochiolitis
Ipex-Like
title_short Folicullar bronchiolitis as phenotype associated with CD25 deficiency
title_full Folicullar bronchiolitis as phenotype associated with CD25 deficiency
title_fullStr Folicullar bronchiolitis as phenotype associated with CD25 deficiency
title_full_unstemmed Folicullar bronchiolitis as phenotype associated with CD25 deficiency
title_sort Folicullar bronchiolitis as phenotype associated with CD25 deficiency
dc.creator.none.fl_str_mv Bezrodnik, Liliana
Caldirola, M. S
Seminario, A. G.
Moreira, I.
Gaillard, M. I.
author Bezrodnik, Liliana
author_facet Bezrodnik, Liliana
Caldirola, M. S
Seminario, A. G.
Moreira, I.
Gaillard, M. I.
author_role author
author2 Caldirola, M. S
Seminario, A. G.
Moreira, I.
Gaillard, M. I.
author2_role author
author
author
author
dc.subject.none.fl_str_mv Cd25 Deficiency
Foxp3
Follicular Brochiolitis
Ipex-Like
topic Cd25 Deficiency
Foxp3
Follicular Brochiolitis
Ipex-Like
purl_subject.fl_str_mv https://purl.org/becyt/ford/3.2
https://purl.org/becyt/ford/3
dc.description.none.fl_txt_mv Regulatory T cells (CD4+CD25+FOXp3+) are subsets of T cells involved in the maintenance of peripheral self tolerance by actively suppressing the activation and expansion of autoreactive T cells.1 Signaling through the interleukin-2 receptor (IL-2R) contributes to T-cell tolerance by controlling three important aspects of Treg biology. CD25 is the α-chain of the IL-2R that, in concert with the β-chain and γ-chain, constitutes the complete IL-2R. 2 CD25 only contributes to IL-2 binding affinity but not to the recruitment of signaling molecules. However, its importance in the development of a normal immune response is emphasized by the finding that a truncation mutant of CD25 results in an immunodeficiency in humans characterized by an increased susceptibility to viral, bacterial and fungal infections. 3 In 1997, Sharfe et al. described an infant with severe bacterial, viral, and fungal infections. Counts of autologous T lymphocytes were moderately low, T cells displayed a weak proliferative response to mitogens in vitro, and the patient displayed no rejection of an allogeneic skin graft. However, unlike children with SCID, the patient besides not having circulating T cells, also developed peripheral lymphocytic proliferation and autoimmune primary biliary cirrhosis. 4 We present the first female argentine patient with mutation in CD25 associated with chronic and severe inflammatory lung disease (Follicular bronchiolitis with lymphocyte hyperplasia), eczema and infections. She has no expression of CD25 on CD4+ T cells and extremely low amount of Tregs.
Fil: Bezrodnik, Liliana. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo Gutierrez"; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina
Fil: Caldirola, M. S. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo Gutierrez"; Argentina
Fil: Seminario, A. G.. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo Gutierrez"; Argentina
Fil: Moreira, I.. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo Gutierrez"; Argentina
Fil: Gaillard, M. I.. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo Gutierrez"; Argentina
description Regulatory T cells (CD4+CD25+FOXp3+) are subsets of T cells involved in the maintenance of peripheral self tolerance by actively suppressing the activation and expansion of autoreactive T cells.1 Signaling through the interleukin-2 receptor (IL-2R) contributes to T-cell tolerance by controlling three important aspects of Treg biology. CD25 is the α-chain of the IL-2R that, in concert with the β-chain and γ-chain, constitutes the complete IL-2R. 2 CD25 only contributes to IL-2 binding affinity but not to the recruitment of signaling molecules. However, its importance in the development of a normal immune response is emphasized by the finding that a truncation mutant of CD25 results in an immunodeficiency in humans characterized by an increased susceptibility to viral, bacterial and fungal infections. 3 In 1997, Sharfe et al. described an infant with severe bacterial, viral, and fungal infections. Counts of autologous T lymphocytes were moderately low, T cells displayed a weak proliferative response to mitogens in vitro, and the patient displayed no rejection of an allogeneic skin graft. However, unlike children with SCID, the patient besides not having circulating T cells, also developed peripheral lymphocytic proliferation and autoimmune primary biliary cirrhosis. 4 We present the first female argentine patient with mutation in CD25 associated with chronic and severe inflammatory lung disease (Follicular bronchiolitis with lymphocyte hyperplasia), eczema and infections. She has no expression of CD25 on CD4+ T cells and extremely low amount of Tregs.
publishDate 2014
dc.date.none.fl_str_mv 2014-01-03
dc.type.none.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
http://purl.org/coar/resource_type/c_6501
info:ar-repo/semantics/articulo
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv http://hdl.handle.net/11336/3694
Bezrodnik, Liliana; Caldirola, M. S; Seminario, A. G.; Moreira, I.; Gaillard, M. I.; Folicullar bronchiolitis as phenotype associated with CD25 deficiency; Wiley; Clinical and Experimental Immunology; 175; 2; 3-1-2014; 227-234
0009-9104
url http://hdl.handle.net/11336/3694
identifier_str_mv Bezrodnik, Liliana; Caldirola, M. S; Seminario, A. G.; Moreira, I.; Gaillard, M. I.; Folicullar bronchiolitis as phenotype associated with CD25 deficiency; Wiley; Clinical and Experimental Immunology; 175; 2; 3-1-2014; 227-234
0009-9104
dc.language.none.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv info:eu-repo/semantics/altIdentifier/url/http://onlinelibrary.wiley.com/doi/10.1111/cei.12214/abstract
info:eu-repo/semantics/altIdentifier/doi/DOI:10.1111/cei.12214
info:eu-repo/semantics/altIdentifier/issn/0009-9104
info:eu-repo/semantics/altIdentifier/url/http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3892414/
dc.rights.none.fl_str_mv info:eu-repo/semantics/openAccess
https://creativecommons.org/licenses/by-nc-sa/2.5/ar/
eu_rights_str_mv openAccess
rights_invalid_str_mv https://creativecommons.org/licenses/by-nc-sa/2.5/ar/
dc.format.none.fl_str_mv application/pdf
application/pdf
dc.publisher.none.fl_str_mv Wiley
publisher.none.fl_str_mv Wiley
dc.source.none.fl_str_mv reponame:CONICET Digital (CONICET)
instname:Consejo Nacional de Investigaciones Científicas y Técnicas
reponame_str CONICET Digital (CONICET)
collection CONICET Digital (CONICET)
instname_str Consejo Nacional de Investigaciones Científicas y Técnicas
repository.name.fl_str_mv CONICET Digital (CONICET) - Consejo Nacional de Investigaciones Científicas y Técnicas
repository.mail.fl_str_mv dasensio@conicet.gov.ar; lcarlino@conicet.gov.ar
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score 13.13397

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