The Experts below are selected from a list of 165 Experts worldwide ranked by ideXlab platform
Pamela J Bjorkman - One of the best experts on this subject based on the ideXlab platform.
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fc receptors and their interactions with Immunoglobulins
Annual Review of Cell and Developmental Biology, 1996Co-Authors: Malini Raghavan, Pamela J BjorkmanAbstract:Receptors for the Fc domain of Immunoglobulins play an important role in immune defense. There are two well-defined functional classes of mammalian receptors. One class of receptors transports Immunoglobulins across epithelial tissues to their main sites of action. This class includes the neonatal Fc receptor (FcRn), which transports immunoglobulin G (IgG), and the polymeric immunoglobulin receptor (pIgR), which transports immunoglobulin A (IgA) and immunoglobulin M (IgM). Another class of receptors present on the surfaces of effector cells triggers various biological responses upon binding antibody-antigen complexes. Of these, the IgG receptors (FcγR) and immunoglobulin E (IgE) receptors (FceR) are the best characterized. The biological responses elicited include antibody-dependent, cell-mediated cytotoxicity, phagocytosis, release of inflammatory mediators, and regulation of lymphocyte proliferation and differentiation. We summarize the current knowledge of the structures and functions of FcRn, pIgR, and the FcγR and FceRI proteins, concentrating on the interactions of the extracellular portions of these receptors with Immunoglobulins.
A. J. Hunter - One of the best experts on this subject based on the ideXlab platform.
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Recurrent Sinopulmonary Infections in a Patient Whose HIV Masked Common Variable Immunodeficiency
Journal of General Internal Medicine, 2020Co-Authors: D. Gollapudi, M. O’donnell, M. Nesmith, K. Kent, A. J. HunterAbstract:It is generally accepted that persons infected with human immunodeficiency virus (HIV) are at an increased risk of infection due to direct destruction of CD4^+ lymphocytes and subsequently impaired cell-mediated immunity. Typically, HIV infection is associated with immunoglobulin elevations, but quantitative deficiencies in Immunoglobulins have also been rarely described. We present an unusual case of common variable immunodeficiency (CVID) in a HIV-positive patient with recurrent severe respiratory infections. We review epidemiology, clinical presentation, and treatment of primary immunoglobulin deficiency. We also review the relationship between immunoglobulin deficiency and HIV and highlight the importance of recognizing the coexistence of two distinct immunodeficiency syndromes.
Dennis R. Burton - One of the best experts on this subject based on the ideXlab platform.
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Human antibody-Fc receptor interactions illuminated by crystal structures
Nature Reviews Immunology, 2004Co-Authors: Jenny M. Woof, Dennis R. BurtonAbstract:Immunoglobulins couple the recognition of invading pathogens with the triggering of potent effector mechanisms for pathogen elimination. Different immunoglobulin classes trigger different effector mechanisms through interaction of immunoglobulin Fc regions with specific Fc receptors (FcRs) on immune cells. Here, we review the structural information that is emerging on three human immunoglobulin classes and their FcRs. New insights are provided, including an understanding of the antibody conformational adjustments that are required to bring effector cell and target cell membranes sufficiently close for efficient killing and signal transduction to occur. The results might also open up new possibilities for the design of therapeutic antibodies.
Russell E Ware - One of the best experts on this subject based on the ideXlab platform.
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childhood autoimmune cytopenia secondary to unsuspected common variable immunodeficiency
The Journal of Pediatrics, 2003Co-Authors: Matthew M Heeney, Sherri A Zimmerman, Russell E WareAbstract:Immune thrombocytopenic purpura and autoimmune hemolytic anemia are typically idiopathic processes without underlying systemic illness. Four children with autoimmune cytopenia had low immunoglobulin levels that led to the diagnosis of common variable immunodeficiency. Routine screening of Immunoglobulins is suggested for children with chronic or recurrent immune thrombocytopenic purpura and autoimmune hemolytic anemia.
Michel Cogne - One of the best experts on this subject based on the ideXlab platform.
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Animal models of monoclonal immunoglobulin-related renal diseases
Nature Reviews Nephrology, 2018Co-Authors: Christophe Sirac, Vincent Javaugue, Sebastien Bender, Guillermo A. Herrera, Paul W. Sanders, Vecihi Batuman, Maria Victoria Ayala, Jiamin Teng, Elba A. Turbat-herrera, Michel CogneAbstract:Monoclonal immunoglobulin deposition produces complex diseases with heterogeneous phenotypes, making it difficult to identify the underlying mechanisms of immunoglobulin aggregation and deposition. In this Review, the authors discuss animal models of various human immunoglobulin deposition diseases, and each model's drawbacks and contributions to our understanding of these diseases. The renal deposition of monoclonal Immunoglobulins can cause severe renal complications in patients with B cell and plasma cell lymphoproliferative disorders. The overproduction of a structurally unique immunoglobulin can contribute to the abnormal propensity of monoclonal Immunoglobulins to aggregate and deposit in specific organs. A wide range of renal diseases can occur in multiple myeloma or monoclonal gammopathy of renal significance, including tubular and glomerular disorders with organized or unorganized immunoglobulin deposits. The development of reliable experimental models is challenging owing to the inherent variability of Immunoglobulins and the heterogeneity of the pathologies they produce. However, although imperfect, animal models are invaluable tools to understand the molecular pathogenesis of these diseases, and advances in creating genetically modified animals might provide novel approaches to evaluate innovative therapeutic interventions. We discuss the strategies employed to reproduce human monoclonal immunoglobulin-induced kidney lesions in animal models, and we highlight their advantages and shortcomings. We also discuss how these models have affected the management of these deposition diseases and might do so in the future. Finally, we discuss hypotheses that explain some limitations of the various models, and how these models might improve our understanding of other nephropathies without immunoglobulin involvement that have similar pathogenic mechanisms. Numerous renal diseases occur owing to the deposition of a monoclonal immunoglobulin, including multiple myeloma and monoclonal gammopathy of renal significance Understanding the molecular pathogenesis of human immunoglobulin deposition diseases and testing new therapeutic strategies requires relevant animal models, which is a challenge owing to the heterogeneity of these diseases Models based on the injection of purified human Immunoglobulins and on tumour grafts that produce the monoclonal immunoglobulin have revealed several early pathogenic events in immunoglobulin deposition and demonstrated the efficacy of innovative therapeutic agents Advances in transgenic techniques have allowed the creation of mouse models that faithfully reproduce the human diseases and have aided in unravelling the pathogenic mechanisms of monoclonal immunoglobulin deposition Animal models are invaluable tools to study the process of deposition and to explore the direct toxicity of monoclonal Immunoglobulins in tissues and immunoglobulin-producing plasma cells
