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.
Keywords:
B-cell receptor; T-cell receptor; common variable immunodeficiency; defective signaling; toll-like receptor.
MeSH terms
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B-Lymphocytes / immunology
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B-Lymphocytes / metabolism*
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Cell Differentiation / immunology
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Cell Proliferation
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Common Variable Immunodeficiency / diagnosis*
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Common Variable Immunodeficiency / etiology
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Common Variable Immunodeficiency / metabolism*
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DNA Repair / immunology
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Humans
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Lymphocyte Activation / immunology*
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Lymphocyte Specific Protein Tyrosine Kinase p56(lck) / genetics
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Lymphocyte Specific Protein Tyrosine Kinase p56(lck) / immunology
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Lymphocyte Specific Protein Tyrosine Kinase p56(lck) / metabolism
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Phosphorylation / immunology
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Receptors, Antigen, B-Cell / immunology
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Receptors, Antigen, B-Cell / metabolism
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Receptors, Antigen, T-Cell / immunology
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Receptors, Antigen, T-Cell / metabolism
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Receptors, IgG / immunology
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Signal Transduction / immunology*
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T-Lymphocytes / immunology
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T-Lymphocytes / metabolism*
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Toll-Like Receptors / immunology
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Toll-Like Receptors / metabolism
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ZAP-70 Protein-Tyrosine Kinase / immunology
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ZAP-70 Protein-Tyrosine Kinase / metabolism
Substances
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Receptors, Antigen, B-Cell
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Receptors, Antigen, T-Cell
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Receptors, IgG
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Toll-Like Receptors
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Lymphocyte Specific Protein Tyrosine Kinase p56(lck)
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ZAP-70 Protein-Tyrosine Kinase