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Educational paper: Defects in number and function of neutrophilic granulocytes causing primary immunodeficiency.

van den Berg JM, Kuijpers TW - Eur. J. Pediatr. (2011)

Bottom Line: The neutrophilic granulocyte (neutrophil) is the most important cellular component of the innate immune system.A total absence of neutrophils or a significant decrease in their number leads to severe immunodeficiency.In order to be neutralized, this pathogen has to be recognized, phagocytosed, and destroyed by lytic enzymes contained in the neutrophil's granules and reactive oxygen species formed by the enzyme complex NADPH oxidase.

View Article: PubMed Central - PubMed

Affiliation: Dept of Pediatric Hematology, Immunology and Infectious Diseases, Emma Children's Hospital, Academic Medical Centre, University of Amsterdam, Room H7-214, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands. j.m.vandenberg@amc.nl

ABSTRACT
The neutrophilic granulocyte (neutrophil) is the most important cellular component of the innate immune system. A total absence of neutrophils or a significant decrease in their number leads to severe immunodeficiency. A mature neutrophil, released from the bone marrow, should be able to migrate from the blood towards the tissues, following a chemotactic gradient to a pathogen. In order to be neutralized, this pathogen has to be recognized, phagocytosed, and destroyed by lytic enzymes contained in the neutrophil's granules and reactive oxygen species formed by the enzyme complex NADPH oxidase. Rare genetic defects leading to the loss of each one of these biological properties of the neutrophil have been described and are associated with immunodeficiency. This review provides a summary of the normal development and biological functions of neutrophils and describes the diseases caused by defects in neutrophil number and function.

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Related in: MedlinePlus

Neutrophil killing. A microorganism is opsonized with immunoglobulins and complement and thus recognized by a neutrophil. The pathogen is then engulfed (phagocytosis) and is digested within the phagosome by lytic enzymes stored in the neutrophil's granules and toxic oxygen radicals formed by the NADPH oxidase
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Fig5: Neutrophil killing. A microorganism is opsonized with immunoglobulins and complement and thus recognized by a neutrophil. The pathogen is then engulfed (phagocytosis) and is digested within the phagosome by lytic enzymes stored in the neutrophil's granules and toxic oxygen radicals formed by the NADPH oxidase

Mentions: Phagocytosis is the initial step which allows neutrophils to destroy an invasive pathogen. In order to be phagocytosed, a pathogen needs to be opsonised. Immunoglobulins, which bind specifically to their antigens, and complement factors, which bind aspecifically to conserved microbial motifs, provide an anchor for their respective receptors on the neutrophil surface. After phagocytosis, the process of pathogen killing starts immediately. The combined activity of an active enzyme complex that is able to produce toxic oxygen radicals as well as the release of various lytic proteins which are stored in the granules (elastase, cathepsin G, defensins) are required to eliminate invading pathogens [13] (Fig. 5).Fig. 5


Educational paper: Defects in number and function of neutrophilic granulocytes causing primary immunodeficiency.

van den Berg JM, Kuijpers TW - Eur. J. Pediatr. (2011)

Neutrophil killing. A microorganism is opsonized with immunoglobulins and complement and thus recognized by a neutrophil. The pathogen is then engulfed (phagocytosis) and is digested within the phagosome by lytic enzymes stored in the neutrophil's granules and toxic oxygen radicals formed by the NADPH oxidase
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC3197933&req=5

Fig5: Neutrophil killing. A microorganism is opsonized with immunoglobulins and complement and thus recognized by a neutrophil. The pathogen is then engulfed (phagocytosis) and is digested within the phagosome by lytic enzymes stored in the neutrophil's granules and toxic oxygen radicals formed by the NADPH oxidase
Mentions: Phagocytosis is the initial step which allows neutrophils to destroy an invasive pathogen. In order to be phagocytosed, a pathogen needs to be opsonised. Immunoglobulins, which bind specifically to their antigens, and complement factors, which bind aspecifically to conserved microbial motifs, provide an anchor for their respective receptors on the neutrophil surface. After phagocytosis, the process of pathogen killing starts immediately. The combined activity of an active enzyme complex that is able to produce toxic oxygen radicals as well as the release of various lytic proteins which are stored in the granules (elastase, cathepsin G, defensins) are required to eliminate invading pathogens [13] (Fig. 5).Fig. 5

Bottom Line: The neutrophilic granulocyte (neutrophil) is the most important cellular component of the innate immune system.A total absence of neutrophils or a significant decrease in their number leads to severe immunodeficiency.In order to be neutralized, this pathogen has to be recognized, phagocytosed, and destroyed by lytic enzymes contained in the neutrophil's granules and reactive oxygen species formed by the enzyme complex NADPH oxidase.

View Article: PubMed Central - PubMed

Affiliation: Dept of Pediatric Hematology, Immunology and Infectious Diseases, Emma Children's Hospital, Academic Medical Centre, University of Amsterdam, Room H7-214, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands. j.m.vandenberg@amc.nl

ABSTRACT
The neutrophilic granulocyte (neutrophil) is the most important cellular component of the innate immune system. A total absence of neutrophils or a significant decrease in their number leads to severe immunodeficiency. A mature neutrophil, released from the bone marrow, should be able to migrate from the blood towards the tissues, following a chemotactic gradient to a pathogen. In order to be neutralized, this pathogen has to be recognized, phagocytosed, and destroyed by lytic enzymes contained in the neutrophil's granules and reactive oxygen species formed by the enzyme complex NADPH oxidase. Rare genetic defects leading to the loss of each one of these biological properties of the neutrophil have been described and are associated with immunodeficiency. This review provides a summary of the normal development and biological functions of neutrophils and describes the diseases caused by defects in neutrophil number and function.

Show MeSH
Related in: MedlinePlus