Follicular T cells from smB(-) common Variable immunodeficiency Patients are skewed Toward a Th1 Phenotype

Germinal center follicular T helper (GCTfh) cells are essential players in the differentiation of B cells. Circulating follicular T helper (cTfh) cells share phenotypic and functional properties with GCTfh cells. Distinct subpopulations of cTfh with different helper capabilities toward B cells can b...

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Authors: Cunill Monjo, Vanesa, Clemente, Antonio, Lanio, Nallibe, Barcelo, Carla, Andreu, Valero, Pons De Ves, Jaime, Ferrer Balaguer, Juana Maria
Format: article
Publication Date:2017
Country:España
Institution:Conselleria de Salut i Consum del Govern de les Illes Balears
Repository:Docusalut
Language:English
OAI Identifier:oai:docusalut.com:20.500.13003/9941
Online Access:https://hdl.handle.net/20.500.13003/9941
Access Level:Open access
Keyword:B cells
CVID
follicular T helper cells
regulatory follicular T cells
Th17.1 cells
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spelling Follicular T cells from smB(-) common Variable immunodeficiency Patients are skewed Toward a Th1 PhenotypeCunill Monjo, VanesaClemente, AntonioLanio, NallibeBarcelo, CarlaAndreu, ValeroPons De Ves, JaimeFerrer Balaguer, Juana MariaB cellsCVIDfollicular T helper cellsregulatory follicular T cellsTh17.1 cellsGerminal center follicular T helper (GCTfh) cells are essential players in the differentiation of B cells. Circulating follicular T helper (cTfh) cells share phenotypic and functional properties with GCTfh cells. Distinct subpopulations of cTfh with different helper capabilities toward B cells can be identified: cTfh1 (CXCR3(+)CCR6(-)), cTfh2 (CXCR3(-)CCR6(-)), and cTfh17 (CXCR3(-)CCR6+). Alterations in cTfh function and/or distribution have been associated with autoimmunity, infectious diseases, and more recently, with several monogenic immunodeficiencies. Common variable immunodeficiency (CVID) disease is the commonest symptomatic primary immunodeficiency with a genetic cause identified in only 2-10% of patients. Although a heterogeneous disease, most patients show a characteristic defective B cell differentiation into memory B cells or antibody-secreting cells. We investigated if alterations in CVID cTfh cells frequency or distribution into cTfh1, cTfh2, and cTfh17 subpopulations and regulatory follicular T (Tfr) cells could be related to defects in CVID B cells. We found increased percentages of cTfh exhibiting higher programmed death-1 expression and altered subpopulations distribution in smB(-) CVID patients. In contrast to smB(+) patients and controls, cTfh from smB-CVID patients show increased cTfh1 and decreased cTfh17 subpopulation percentages and increased CXCR3(+)CCR6(+) cTfh, a population analogous to the recently described pathogenic Th17.1. Moreover, Tfr cells are remarkably decreased only in smB-CVID patients. In conclusion, increased cTfh17.1 and cTfh1/cTfh17 ratio in CVID patients could influence B cell fate in smB-CVID patients, with a more compromised B cell compartment, and the decrease in Tfr cells may lead to high risk of autoimmune conditions in CVID patients.Frontiers Media Sa20172017-02-2720172017-02-27research articlehttp://purl.org/coar/resource_type/c_2df8fbb1info:eu-repo/semantics/articleapplication/pdfhttps://hdl.handle.net/20.500.13003/9941reponame:Docusalutinstname:Conselleria de Salut i Consum del Govern de les Illes BalearsInglésengopen accesshttp://purl.org/coar/access_right/c_abf2Attribution 4.0 Internationalhttp://creativecommons.org/licenses/by/4.0/info:eu-repo/semantics/openAccessoai:docusalut.com:20.500.13003/99412026-06-22T12:44:07Z
dc.title.none.fl_str_mv Follicular T cells from smB(-) common Variable immunodeficiency Patients are skewed Toward a Th1 Phenotype
title Follicular T cells from smB(-) common Variable immunodeficiency Patients are skewed Toward a Th1 Phenotype
spellingShingle Follicular T cells from smB(-) common Variable immunodeficiency Patients are skewed Toward a Th1 Phenotype
Cunill Monjo, Vanesa
B cells
CVID
follicular T helper cells
regulatory follicular T cells
Th17.1 cells
title_short Follicular T cells from smB(-) common Variable immunodeficiency Patients are skewed Toward a Th1 Phenotype
title_full Follicular T cells from smB(-) common Variable immunodeficiency Patients are skewed Toward a Th1 Phenotype
title_fullStr Follicular T cells from smB(-) common Variable immunodeficiency Patients are skewed Toward a Th1 Phenotype
title_full_unstemmed Follicular T cells from smB(-) common Variable immunodeficiency Patients are skewed Toward a Th1 Phenotype
title_sort Follicular T cells from smB(-) common Variable immunodeficiency Patients are skewed Toward a Th1 Phenotype
dc.creator.none.fl_str_mv Cunill Monjo, Vanesa
Clemente, Antonio
Lanio, Nallibe
Barcelo, Carla
Andreu, Valero
Pons De Ves, Jaime
Ferrer Balaguer, Juana Maria
author Cunill Monjo, Vanesa
author_facet Cunill Monjo, Vanesa
Clemente, Antonio
Lanio, Nallibe
Barcelo, Carla
Andreu, Valero
Pons De Ves, Jaime
Ferrer Balaguer, Juana Maria
author_role author
author2 Clemente, Antonio
Lanio, Nallibe
Barcelo, Carla
Andreu, Valero
Pons De Ves, Jaime
Ferrer Balaguer, Juana Maria
author2_role author
author
author
author
author
author
dc.contributor.none.fl_str_mv
dc.subject.none.fl_str_mv B cells
CVID
follicular T helper cells
regulatory follicular T cells
Th17.1 cells
topic B cells
CVID
follicular T helper cells
regulatory follicular T cells
Th17.1 cells
description Germinal center follicular T helper (GCTfh) cells are essential players in the differentiation of B cells. Circulating follicular T helper (cTfh) cells share phenotypic and functional properties with GCTfh cells. Distinct subpopulations of cTfh with different helper capabilities toward B cells can be identified: cTfh1 (CXCR3(+)CCR6(-)), cTfh2 (CXCR3(-)CCR6(-)), and cTfh17 (CXCR3(-)CCR6+). Alterations in cTfh function and/or distribution have been associated with autoimmunity, infectious diseases, and more recently, with several monogenic immunodeficiencies. Common variable immunodeficiency (CVID) disease is the commonest symptomatic primary immunodeficiency with a genetic cause identified in only 2-10% of patients. Although a heterogeneous disease, most patients show a characteristic defective B cell differentiation into memory B cells or antibody-secreting cells. We investigated if alterations in CVID cTfh cells frequency or distribution into cTfh1, cTfh2, and cTfh17 subpopulations and regulatory follicular T (Tfr) cells could be related to defects in CVID B cells. We found increased percentages of cTfh exhibiting higher programmed death-1 expression and altered subpopulations distribution in smB(-) CVID patients. In contrast to smB(+) patients and controls, cTfh from smB-CVID patients show increased cTfh1 and decreased cTfh17 subpopulation percentages and increased CXCR3(+)CCR6(+) cTfh, a population analogous to the recently described pathogenic Th17.1. Moreover, Tfr cells are remarkably decreased only in smB-CVID patients. In conclusion, increased cTfh17.1 and cTfh1/cTfh17 ratio in CVID patients could influence B cell fate in smB-CVID patients, with a more compromised B cell compartment, and the decrease in Tfr cells may lead to high risk of autoimmune conditions in CVID patients.
publishDate 2017
dc.date.none.fl_str_mv 2017
2017-02-27
2017
2017-02-27
dc.type.none.fl_str_mv research article
http://purl.org/coar/resource_type/c_2df8fbb1
dc.type.openaire.fl_str_mv info:eu-repo/semantics/article
format article
dc.identifier.none.fl_str_mv https://hdl.handle.net/20.500.13003/9941
url https://hdl.handle.net/20.500.13003/9941
dc.language.none.fl_str_mv Inglés
eng
language_invalid_str_mv Inglés
language eng
dc.rights.none.fl_str_mv open access
http://purl.org/coar/access_right/c_abf2
Attribution 4.0 International
http://creativecommons.org/licenses/by/4.0/
dc.rights.openaire.fl_str_mv info:eu-repo/semantics/openAccess
rights_invalid_str_mv open access
http://purl.org/coar/access_right/c_abf2
Attribution 4.0 International
http://creativecommons.org/licenses/by/4.0/
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.publisher.none.fl_str_mv Frontiers Media Sa
publisher.none.fl_str_mv Frontiers Media Sa
dc.source.none.fl_str_mv reponame:Docusalut
instname:Conselleria de Salut i Consum del Govern de les Illes Balears
instname_str Conselleria de Salut i Consum del Govern de les Illes Balears
reponame_str Docusalut
collection Docusalut
repository.name.fl_str_mv
repository.mail.fl_str_mv
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score 15,811543

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