Por favor, use este identificador para citar o enlazar este Item:http://hdl.handle.net/20.500.12105/20412
Título
Follicular T cells from smB(-) common Variable immunodeficiency Patients are skewed Toward a Th1 Phenotype
Autor(es)
Fecha de publicación
2017-02-27
Cita
Cunill Monjo V, Clemente A, Lanio N, Barcelo C, Andreu Matillas V, Pons De Ves J, et al. Follicular T cells from smB(-) common Variable immunodeficiency Patients are skewed Toward a Th1 Phenotype. Front Immunol. 2017 Feb 27;8:174.
Idioma
Inglés
Tipo de documento
research article
Resumen
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.
Palabras clave
Versión en línea
DOI
Aparece en las colecciones
Acceso a texto completo