Current Concepts of Hyperinflammation in Chronic Granulomatous Disease
Chronic granulomatous disease (CGD) is the most common inherited disorder of phagocytic functions, caused by genetic defects in the leukocyte nicotinamide dinucleotide phosphate (NADPH) oxidase. Consequently, CGD phagocytes are impaired in destroying phagocytosed microorganisms, rendering the patien...
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| Format: | Article |
| Language: | English |
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Wiley
2012-01-01
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| Series: | Clinical and Developmental Immunology |
| Online Access: | http://dx.doi.org/10.1155/2012/252460 |
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| author | Nikolaus Rieber Andreas Hector Taco Kuijpers Dirk Roos Dominik Hartl |
| author_facet | Nikolaus Rieber Andreas Hector Taco Kuijpers Dirk Roos Dominik Hartl |
| author_sort | Nikolaus Rieber |
| collection | DOAJ |
| description | Chronic granulomatous disease (CGD) is the most common inherited disorder of phagocytic functions, caused by genetic defects in the leukocyte nicotinamide dinucleotide phosphate (NADPH) oxidase. Consequently, CGD phagocytes are impaired in destroying phagocytosed microorganisms, rendering the patients susceptible to bacterial and fungal infections. Besides this immunodeficiency, CGD patients suffer from various autoinflammatory symptoms, such as granuloma formation in the skin or urinary tract and Crohn-like colitis. Owing to improved antimicrobial treatment strategies, the majority of CGD patients reaches adulthood, yet the autoinflammatory manifestations become more prominent by lack of causative treatment options. The underlying pathomechanisms driving hyperinflammatory reactions in CGD are poorly understood, but recent studies implicate reduced neutrophil apoptosis and efferocytosis, dysbalanced innate immune receptors, altered T-cell surface redox levels, induction of Th17 cells, the enzyme indolamine-2,3-dioxygenase (IDO), impaired Nrf2 activity, and inflammasome activation. Here we discuss immunological mechanisms of hyperinflammation and their potential therapeutic implications in CGD. |
| format | Article |
| id | doaj-art-8ca2b01fd5c54e90b6d98c7a2d318ddc |
| institution | Kabale University |
| issn | 1740-2522 1740-2530 |
| language | English |
| publishDate | 2012-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Clinical and Developmental Immunology |
| spelling | doaj-art-8ca2b01fd5c54e90b6d98c7a2d318ddc2025-02-03T07:25:57ZengWileyClinical and Developmental Immunology1740-25221740-25302012-01-01201210.1155/2012/252460252460Current Concepts of Hyperinflammation in Chronic Granulomatous DiseaseNikolaus Rieber0Andreas Hector1Taco Kuijpers2Dirk Roos3Dominik Hartl4Children's Hospital, University of Tübingen, 72076 Tübingen, GermanyChildren's Hospital, University of Tübingen, 72076 Tübingen, GermanySanquin Research Institute, Landsteiner Laboratory, 1006 AD Amsterdam, The NetherlandsSanquin Research Institute, Landsteiner Laboratory, 1006 AD Amsterdam, The NetherlandsChildren's Hospital, University of Tübingen, 72076 Tübingen, GermanyChronic granulomatous disease (CGD) is the most common inherited disorder of phagocytic functions, caused by genetic defects in the leukocyte nicotinamide dinucleotide phosphate (NADPH) oxidase. Consequently, CGD phagocytes are impaired in destroying phagocytosed microorganisms, rendering the patients susceptible to bacterial and fungal infections. Besides this immunodeficiency, CGD patients suffer from various autoinflammatory symptoms, such as granuloma formation in the skin or urinary tract and Crohn-like colitis. Owing to improved antimicrobial treatment strategies, the majority of CGD patients reaches adulthood, yet the autoinflammatory manifestations become more prominent by lack of causative treatment options. The underlying pathomechanisms driving hyperinflammatory reactions in CGD are poorly understood, but recent studies implicate reduced neutrophil apoptosis and efferocytosis, dysbalanced innate immune receptors, altered T-cell surface redox levels, induction of Th17 cells, the enzyme indolamine-2,3-dioxygenase (IDO), impaired Nrf2 activity, and inflammasome activation. Here we discuss immunological mechanisms of hyperinflammation and their potential therapeutic implications in CGD.http://dx.doi.org/10.1155/2012/252460 |
| spellingShingle | Nikolaus Rieber Andreas Hector Taco Kuijpers Dirk Roos Dominik Hartl Current Concepts of Hyperinflammation in Chronic Granulomatous Disease Clinical and Developmental Immunology |
| title | Current Concepts of Hyperinflammation in Chronic Granulomatous Disease |
| title_full | Current Concepts of Hyperinflammation in Chronic Granulomatous Disease |
| title_fullStr | Current Concepts of Hyperinflammation in Chronic Granulomatous Disease |
| title_full_unstemmed | Current Concepts of Hyperinflammation in Chronic Granulomatous Disease |
| title_short | Current Concepts of Hyperinflammation in Chronic Granulomatous Disease |
| title_sort | current concepts of hyperinflammation in chronic granulomatous disease |
| url | http://dx.doi.org/10.1155/2012/252460 |
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