title: Evaluation of Known Defective Signaling-Associated Molecules in Patients Who Primarily Diagnosed as Common Variable Immunodeficiency.
abstract: Common variable immunodeficiency (CVID) is a primary immunodeficiency disease, associated with defective antibody production and recurrent infections. Several defective signaling-associated molecules related to B-cell receptor, T-cell receptor, toll-like receptor, and other immune cell-associated receptors have been identified so far. In this review, defects of cell signaling associated molecules at three levels of surface cytoplasmic, and nuclear molecules are highlighted in patients who primarily diagnosed as CVID. It could be suggested that impaired cell signaling may be involved in the pathogenesis of CVID.
authors.affiliation: a Department of Immunology, School of Medicine , Isfahan University of Medical Sciences , Isfahan , Iran.|b Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center , Tehran University of Medical Science , Tehran , Iran.
authors.forename: Reza
authors.initials: R
authors.lastname: Yazdani
authors.affiliation: b Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center , Tehran University of Medical Science , Tehran , Iran.|c Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institutet at the Karolinska University Hospital Huddinge , Stockholm , Sweden.
authors.forename: Hassan
authors.initials: H
authors.lastname: Abolhassani
authors.affiliation: b Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center , Tehran University of Medical Science , Tehran , Iran.|d Universal Scientific Education and Research Network (USERN) , Tehran , Iran.|e Department of Immunology, School of Medicine , Tehran University of Medical Science , Tehran , Iran.
authors.forename: Nima
authors.initials: N
authors.lastname: Rezaei
authors.affiliation: f Imam Hassan Mojtaba Hospital , Alborz University of Medical Sciences , Karaj , Iran.
authors.forename: Gholamreza
authors.initials: G
authors.lastname: Azizi
authors.affiliation: c Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institutet at the Karolinska University Hospital Huddinge , Stockholm , Sweden.
authors.forename: Lennart
authors.initials: L
authors.lastname: Hammarström
authors.affiliation: b Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center , Tehran University of Medical Science , 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; D049109:Cell Proliferation; D017074:Common Variable Immunodeficiency / Q000175:diagnosis* / Q000209:etiology / Q000378:metabolism*; D004260:DNA Repair / Q000276:immunology; D006801:Humans; D008213:Lymphocyte Activation / Q000276:immunology*; D019860:Lymphocyte Specific Protein Tyrosine Kinase p56(lck) / Q000235:genetics / Q000276:immunology / Q000378:metabolism; D010766:Phosphorylation / Q000276:immunology; D011947:Receptors, Antigen, B-Cell / Q000276:immunology / Q000378:metabolism; D011948:Receptors, Antigen, T-Cell / Q000276:immunology / Q000378:metabolism; D017452:Receptors, IgG / Q000276:immunology; D015398:Signal Transduction / Q000276:immunology*; D013601:T-Lymphocytes / Q000276:immunology / Q000378:metabolism*; D051193:Toll-Like Receptors / Q000276:immunology / Q000378:metabolism; D051746:ZAP-70 Protein-Tyrosine Kinase / Q000276:immunology / Q000378:metabolism
chemical_list: D011947:Receptors, Antigen, B-Cell; D011948:Receptors, Antigen, T-Cell; D017452:Receptors, IgG; D051193:Toll-Like Receptors; D019860:Lymphocyte Specific Protein Tyrosine Kinase p56(lck); D051746:ZAP-70 Protein-Tyrosine Kinase
keywords: B-cell receptor; T-cell receptor; common variable immunodeficiency; defective signaling; toll-like receptor