Immunological and genetic bases of new primary immunodeficiencies |
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Authors: | Maródi László Notarangelo Luigi D |
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Institution: | Department of Infectious and Pediatric Immunology, University of Debrecen Medical and Health Science Center, H-4,012 Debrecen, Hungary. lmarodi@dote.hu |
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Abstract: | Since 1952, when congenital agammaglobulinaemia was described by Bruton, the characterization of genetically defined immunodeficiencies in humans has been crucial for a better understanding of the biology of the innate and adaptive immune responses. This Review focuses on the characterization of new primary immunodeficiencies and disease-related genes. A series of primary defects of innate immunity have recently been discovered and are discussed here. Moreover, new defects in pre-B-cell and B-cell differentiation and antibody maturation are summarized and recently discovered monogenic immunodeficiencies that disturb the homeostasis of both the innate and the adaptive immune systems are discussed. |
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