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Human and animal models of V(D)J recombination deficiency |
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| Authors: | de Villartay Jean-Pierre Poinsignon Catherine de Chasseval Regina Buck Dietke Le Guyader Gwenaël Villey Isabelle |
| Affiliation: | 1. Department of Movement, Human and Health Sciences, Italian University Sport and Movement “Foro Italico”, Rome, Italy;2. Research Institute for Neuroscience, Education and Didactics, Patrizio Paoletti Foundation for Development and Communication, Assisi, Italy;3. Bar-Ilan University, Ramat-Gan, Israel;1. Division of Immunopathology, Department of Pathophysiology and Allergy Research, Center for Pathophysiology, Infectiology and Immunology, Medical University of Vienna, Vienna, Austria;2. Airway Disease Infection Section, National Heart & Lung Institute, Imperial College London, UK;3. MRC and Asthma UK Centre in Allergic Mechanisms of Asthma, London, UK |
| Abstract: | V(D)J recombination not only comprises the molecular mechanism that insures diversity of the immune system but also constitutes a critical checkpoint in the developmental program of B and T lymphocytes. The analysis of human patients with severe combined immune deficiency (SCID) has enabled (and will enable in the future) the discovery of important factors involved in this reaction. The finding that the V(D)J recombinase apparatus includes components of the general DNA repair machinery of the cells has provided some new and interesting insights into the role of V(D)J recombination deficiency in the development of lymphoid malignancies, a hypothesis that has been tackled and proven in several animal models. |
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