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Defects in Innate Immunity: Receptors and Signaling Components Publisher



Mahdiabadi S1, 2, 3 ; Rezaei N2, 3, 4
Authors
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Authors Affiliations
  1. 1. School of Medicine, Tehran University of Medical Sciences, Tehran, Iran
  2. 2. Network of Immunity in Infection, Malignancy and Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN), Tehran, Iran
  3. 3. Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran
  4. 4. Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran

Source: Encyclopedia of Infection and Immunity Published:2022


Abstract

The human innate immune system is designed to provide first-line defense against invading pathogens. It is composed of a diverse range of components, a key member of which is pattern recognition receptors. Genetic defects in the signaling pathways and receptors of the innate immune system cause primary immunodeficiencies, resulting in increased susceptibility to serious infections by miscellaneous pathogens, including mycobacteria, viruses, and fungi. The most important so-far discovered defects associated with innate immunity receptors and signaling include defects in the PRRs signaling, defects causing Mendelian susceptibility to mycobacterial diseases, Defects causing WHIM syndrome, and defects causing Epidermodysplasia verruciformis. © 2022 Elsevier Inc. All rights reserved.
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