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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 motility. Activation of endothelial cells leads to rolling of neutrophils in the blood circulation along the endothelial cell lining, mediated by adhesion molecules called selectins, and their sugar ligands. This results in firm adhesion, mediated by the family of adhesion molecules called integrins. These steps are essential for the subsequent migration of neutrophils, between the endothelial cells, towards the inflammatory focus in the tissues beneath. Absence of these adhesion molecules in LAD2 (selectin ligands) and LAD1 (β2 integrins) leads to immunodeficiency because otherwise normal neutrophils are unable to leave the blood stream and reach the inflammatory site
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Fig4: Neutrophil motility. Activation of endothelial cells leads to rolling of neutrophils in the blood circulation along the endothelial cell lining, mediated by adhesion molecules called selectins, and their sugar ligands. This results in firm adhesion, mediated by the family of adhesion molecules called integrins. These steps are essential for the subsequent migration of neutrophils, between the endothelial cells, towards the inflammatory focus in the tissues beneath. Absence of these adhesion molecules in LAD2 (selectin ligands) and LAD1 (β2 integrins) leads to immunodeficiency because otherwise normal neutrophils are unable to leave the blood stream and reach the inflammatory site

Mentions: Leukocytes recognize concentration differences in a gradient of chemotaxins and thus direct their movement towards the source of these agents, i.e., towards the inflammatory site. Apart from the ability to move, binding to endothelial cells is essential for neutrophils to leave the bloodstream. Movement between or through endothelial cells is mediated by adhesion molecules. Further on, neutrophils bind to tissue cells and extracellular matrix components (Fig. 4). These interactions are dynamic because in order to move, after first binding their ligands, adhesion molecules at “the tail of the movement” subsequently must be able to detach. By then binding to other ligands at “the front of the movement,” the neutrophil is able to move on. Of course, these adhesion molecules and their controlled ability to switch on and off (i.e., get activated and deactivated) are tightly regulated [19].Fig. 4


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

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

Neutrophil motility. Activation of endothelial cells leads to rolling of neutrophils in the blood circulation along the endothelial cell lining, mediated by adhesion molecules called selectins, and their sugar ligands. This results in firm adhesion, mediated by the family of adhesion molecules called integrins. These steps are essential for the subsequent migration of neutrophils, between the endothelial cells, towards the inflammatory focus in the tissues beneath. Absence of these adhesion molecules in LAD2 (selectin ligands) and LAD1 (β2 integrins) leads to immunodeficiency because otherwise normal neutrophils are unable to leave the blood stream and reach the inflammatory site
© Copyright Policy
Related In: Results  -  Collection

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

Fig4: Neutrophil motility. Activation of endothelial cells leads to rolling of neutrophils in the blood circulation along the endothelial cell lining, mediated by adhesion molecules called selectins, and their sugar ligands. This results in firm adhesion, mediated by the family of adhesion molecules called integrins. These steps are essential for the subsequent migration of neutrophils, between the endothelial cells, towards the inflammatory focus in the tissues beneath. Absence of these adhesion molecules in LAD2 (selectin ligands) and LAD1 (β2 integrins) leads to immunodeficiency because otherwise normal neutrophils are unable to leave the blood stream and reach the inflammatory site
Mentions: Leukocytes recognize concentration differences in a gradient of chemotaxins and thus direct their movement towards the source of these agents, i.e., towards the inflammatory site. Apart from the ability to move, binding to endothelial cells is essential for neutrophils to leave the bloodstream. Movement between or through endothelial cells is mediated by adhesion molecules. Further on, neutrophils bind to tissue cells and extracellular matrix components (Fig. 4). These interactions are dynamic because in order to move, after first binding their ligands, adhesion molecules at “the tail of the movement” subsequently must be able to detach. By then binding to other ligands at “the front of the movement,” the neutrophil is able to move on. Of course, these adhesion molecules and their controlled ability to switch on and off (i.e., get activated and deactivated) are tightly regulated [19].Fig. 4

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