title: The role of toll-like receptors in B-cell development and immunopathogenesis of common variable immunodeficiency.
abstract: Common variable immunodeficiency (CVID) is the most frequent symptomatic primary immune deficiency and is characterized by hypogammaglobulinemia, defect in specific antibody response and increased susceptibility to recurrent infections, malignancy and autoimmunity. Patients with CVID often have defects in post-antigenic B-cell differentiation, with fewer memory B cells and impaired isotype switching. Toll-like receptors (TLRs) are expressed on various immune cells as key elements of innate and adaptive immunity. TLR signaling in B cells plays multiple roles in cell differentiation and activation, class-switch recombination and cytokine and antibody production. Moreover, recent studies have shown functional alteration of TLRs responses in CVID patients including poor cell proliferation, impaired upregulation of co-stimulatory molecules and failure in cytokine and immunoglobulin production. The purpose of the present review is to discuss the role of TLRs in B-cell development and function as well as their role in the immunopathogenesis of CVID.
authors.affiliation: a Research Center for Immunodeﬁciencies, Pediatrics Center of Excellence, Children's Medical Center , Tehran University of Medical Sciences , Tehran , Iran.
authors.forename: Laleh
authors.initials: L
authors.lastname: Sharifi
authors.affiliation: a Research Center for Immunodeﬁciencies, Pediatrics Center of Excellence, Children's Medical Center , Tehran University of Medical Sciences , Tehran , Iran.|b Department of Immunology, School of Public Health , Tehran University of Medical Sciences , Tehran , Iran.
authors.forename: Abbas
authors.initials: A
authors.lastname: Mirshafiey
authors.affiliation: a Research Center for Immunodeﬁciencies, Pediatrics Center of Excellence, Children's Medical Center , Tehran University of Medical Sciences , Tehran , Iran.|c Department of Immunology, School of Medicine , Tehran University of Medical Sciences , Tehran , Iran.
authors.forename: Nima
authors.initials: N
authors.lastname: Rezaei
authors.affiliation: d Imam Hassan Mojtaba Hospital , Alborz University of Medical Sciences , Karaj , Iran.
authors.forename: Gholamreza
authors.initials: G
authors.lastname: Azizi
authors.affiliation: b Department of Immunology, School of Public Health , Tehran University of Medical Sciences , Tehran , Iran.|e Immunology Department, Faculty of Medical Laboratory Sciences , Usmanu Danfodiyo University , Sokoto , Nigeria.
authors.forename: Kabir
authors.initials: K
authors.lastname: Magaji Hamid
authors.affiliation: c Department of Immunology, School of Medicine , Tehran University of Medical Sciences , Tehran , Iran.
authors.forename: Ali Akbar
authors.initials: AA
authors.lastname: Amirzargar
authors.affiliation: a Research Center for Immunodeﬁciencies, Pediatrics Center of Excellence, Children's Medical Center , Tehran University of Medical Sciences , Tehran , Iran.
authors.forename: Mohammad Hossein
authors.initials: MH
authors.lastname: Asgardoon
authors.affiliation: a Research Center for Immunodeﬁciencies, Pediatrics Center of Excellence, Children's Medical Center , Tehran University of Medical Sciences , Tehran , Iran.
authors.forename: Asghar
authors.initials: A
authors.lastname: Aghamohammadi
mesh_terms: D001402:B-Lymphocytes / Q000276:immunology* / Q000378:metabolism; D002454:Cell Differentiation / Q000276:immunology; D017074:Common Variable Immunodeficiency / Q000276:immunology* / Q000378:metabolism; D016207:Cytokines / Q000276:immunology / Q000378:metabolism; D006801:Humans; D017578:Immunoglobulin Class Switching / Q000276:immunology; D008213:Lymphocyte Activation / Q000276:immunology; D018448:Models, Immunological; D015398:Signal Transduction / Q000276:immunology*; D051193:Toll-Like Receptors / Q000276:immunology* / Q000378:metabolism
chemical_list: D016207:Cytokines; D051193:Toll-Like Receptors
keywords: B cell; Common variable immunodeficiency; Toll-like receptor; hypogammaglobulinemia; isotype switching