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Defects in Intrinsic and Innate Immunity: Receptors and Signaling Components

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Primary Immunodeficiency Diseases

Abstract

The innate immunity is the first line of defense against pathogens, exerting fast and effective responses via preformed receptors. Moreover, this sensitive system puts together signals to induce adaptive host responses. This field has evolved a lot during the recent decades after the discovery of pattern recognition receptors that recognize pathogen specific molecular patterns. The increased understanding of the molecular and cellular components of the innate immune system authorized the dissection of novel human PIDs. The development and testing of a human genetic theory of infectious diseases has resulted in the discovery of single-gene inborn errors of (innate) immunity underlying severe infections in otherwise healthy children.

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Parvaneh, N., Lilic, D., Roesler, J., Niehues, T., Casanova, JL., Picard, C. (2017). Defects in Intrinsic and Innate Immunity: Receptors and Signaling Components. In: Rezaei, N., Aghamohammadi, A., Notarangelo, L. (eds) Primary Immunodeficiency Diseases. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-52909-6_6

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