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Title:
Inflammation and repeated infections in CGD: two sides of a coin | Cellular and Molecular Life Sciences
Description:
Chronic granulomatous disease (CGD) is an uncommon congenital immunodeficiency seen approximately in 1 of 250,000 individuals. It is caused by a profound defect in a burst of oxygen consumption that normally accompanies phagocytosis in all myeloid cells (neutrophils, eosinophils, monocytes, and macrophages). This ârespiratory burstâ involves the catalytic conversion of molecular oxygen to the oxygen free-radical superoxide, which in turn gives rise to hydrogen peroxide, hypochlorous acid, and hydroxyl radicals. These oxygen derivatives play a critical role in the killing of pathogenic bacteria and fungi. As a result of the failure to activate the respiratory burst in their phagocytes, the majority of CGD patients suffer from severe recurrent infections and rather unexplained prolonged inflammatory reactions that may result in granulomatous lesions. Both may cause severe organ dysfunction depending on the tissues involved. Preventive measures as well as rapid (invasive) diagnostic procedures are required to successfully treat CGD. Hematopoietic stem cell transplantation may be a serious option in some of the patients.
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cgd, cells, article, google, scholar, pubmed, cas, ido, cell, nadph, disease, patients, oxidase, activity, chronic, granulomatous, inflammation, inflammatory, mice, macrophages, responses, immune, pphox, activation, production, lkynurenine, van, infection, regulatory, tryptophan, human, immunol, ros, tissue, oxygen, neutrophils, role, aspergillus, defective, response, induction, pathogens, innate, lung, tregs, species, granuloma, romani, kuijpers, superoxide,
Topics {âď¸}
autosomal-dominant hyper-ige syndrome cd4 + cd25 + antigen-specific regulatory vitro-expanded antigen-specific regulatory article download pdf ido-mediated l-tryptophan metabolism cd4 + cd25 + foxp3 + chronic granulomatous disease catalase-deficient aspergillus nidulans augmented nf-κb activation gp 91phox-deficient patients th2 cell-signaling pathways hyper-ige syndrome l-kynurenine suppletion restores natural l-kynurenine distal oxygen free-radical superoxide amino acid l-tryptophan full size image constitutively express il-17r inadequate anti-microbial defense increased nf-κb activation ido converts l-tryptophan zymosan-induced lung inflammation inadequate anti-microbial response ifn-γ amplifies il-6 membrane-bound tgf-β [51] ido product l-kynurenine ido-generated l-kynurenine ifn-γ-exposed monocytes il-17-mediated immune pathway drive chronic inflammation reactive oxygen species van der loos van der poll redox-mediated signaling tight junction proteins privacy choices/manage cookies exaggerated inflammatory responses gp91phox-negative dendritic cells promote chronic inflammation nadph oxidase-deficient p47phox-mutated background innate il-17-producing cells inflammatory immune responses anti-inflammatory agent activate tgf-β exaggerated inflammatory response regulatory pathways exist develop autoimmune diseases tryptophan/kynurenine metabolism anti-inflammatory macrophages
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headline:Inflammation and repeated infections in CGD: two sides of a coin
description:Chronic granulomatous disease (CGD) is an uncommon congenital immunodeficiency seen approximately in 1 of 250,000 individuals. It is caused by a profound defect in a burst of oxygen consumption that normally accompanies phagocytosis in all myeloid cells (neutrophils, eosinophils, monocytes, and macrophages). This ârespiratory burstâ involves the catalytic conversion of molecular oxygen to the oxygen free-radical superoxide, which in turn gives rise to hydrogen peroxide, hypochlorous acid, and hydroxyl radicals. These oxygen derivatives play a critical role in the killing of pathogenic bacteria and fungi. As a result of the failure to activate the respiratory burst in their phagocytes, the majority of CGD patients suffer from severe recurrent infections and rather unexplained prolonged inflammatory reactions that may result in granulomatous lesions. Both may cause severe organ dysfunction depending on the tissues involved. Preventive measures as well as rapid (invasive) diagnostic procedures are required to successfully treat CGD. Hematopoietic stem cell transplantation may be a serious option in some of the patients.
datePublished:2011-11-15T00:00:00Z
dateModified:2011-11-15T00:00:00Z
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pageEnd:15
license:https://creativecommons.org/licenses/by-nc/2.0
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keywords:
Infection
Inflammation
Granuloma
NADPH oxidase
IDO
Tryptophan
Kynurenine
IL-17
Tregs
Cell Biology
Biomedicine
general
Life Sciences
Biochemistry
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headline:Inflammation and repeated infections in CGD: two sides of a coin
description:Chronic granulomatous disease (CGD) is an uncommon congenital immunodeficiency seen approximately in 1 of 250,000 individuals. It is caused by a profound defect in a burst of oxygen consumption that normally accompanies phagocytosis in all myeloid cells (neutrophils, eosinophils, monocytes, and macrophages). This ârespiratory burstâ involves the catalytic conversion of molecular oxygen to the oxygen free-radical superoxide, which in turn gives rise to hydrogen peroxide, hypochlorous acid, and hydroxyl radicals. These oxygen derivatives play a critical role in the killing of pathogenic bacteria and fungi. As a result of the failure to activate the respiratory burst in their phagocytes, the majority of CGD patients suffer from severe recurrent infections and rather unexplained prolonged inflammatory reactions that may result in granulomatous lesions. Both may cause severe organ dysfunction depending on the tissues involved. Preventive measures as well as rapid (invasive) diagnostic procedures are required to successfully treat CGD. Hematopoietic stem cell transplantation may be a serious option in some of the patients.
datePublished:2011-11-15T00:00:00Z
dateModified:2011-11-15T00:00:00Z
pageStart:7
pageEnd:15
license:https://creativecommons.org/licenses/by-nc/2.0
sameAs:https://doi.org/10.1007/s00018-011-0834-z
keywords:
Infection
Inflammation
Granuloma
NADPH oxidase
IDO
Tryptophan
Kynurenine
IL-17
Tregs
Cell Biology
Biomedicine
general
Life Sciences
Biochemistry
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