title: Recent insights into the pathobiology of innate immune deficiencies.
abstract: Primary immunodeficiencies are a heterogeneous group of genetically inherited diseases affecting the innate and adaptive immune systems that confer susceptibility to infection, autoimmunity, and cancer. Innate immunity includes neutrophils, macrophages, dendritic cells, natural killer cells, and natural killer T cells in conjunction with natural barriers (mostly skin and gastrointestinal and respiratory mucosa), as well as antimicrobial agents, opsonins (e.g., complement), and cytokines. Although somewhat primitive, innate immune cells can orchestrate discrete immune responses through the recognition of diverse pathogens by different pattern-recognition receptors. In this review, we discuss the most recent discoveries as well as the already established pathophysiologic mechanisms underlying innate immunity defects associated with primary immunodeficiencies.
authors.affiliation: Infectious Diseases Susceptibility Unit, Laboratory of Host Defenses, National Institute of Allergy and Infectious Diseases, National Institutes of Health, 10 Center Drive, Building 10, CRC 6W-3750, Bethesda, MD 20892-1456, USA. srosenzweig@niaid.nih.gov
authors.forename: Sergio D
authors.initials: SD
authors.lastname: Rosenzweig
authors.forename: Steven M
authors.initials: SM
authors.lastname: Holland
mesh_terms: D000818:Animals; D015551:Autoimmunity / Q000235:genetics; D017074:Common Variable Immunodeficiency / Q000235:genetics / Q000276:immunology* / Q000473:pathology / Q000503:physiopathology*; D030342:Genetic Diseases, Inborn / Q000235:genetics / Q000276:immunology / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D007113:Immunity, Innate; D007962:Leukocytes / Q000276:immunology* / Q000473:pathology; D009928:Organ Specificity / Q000235:genetics