نتایج جستجو برای: cgd

تعداد نتایج: 685  

Journal: :Arteriosclerosis, thrombosis, and vascular biology 2000
E A Kirk M C Dinauer H Rosen A Chait J W Heinecke R C LeBoeuf

Superoxide, the reduced form of molecular oxygen, has been implicated in the genesis of vascular disease. One potential mechanism involves oxidation of low density lipoprotein into an atherogenic particle. A second involves reaction with nitric oxide to generate peroxynitrite, a highly oxidizing intermediate. A third involves regulation of signal transduction in artery wall cells. One well-char...

Journal: :iranian journal of allergy, asthma and immunology 0
payam tabarsi mehdi mirsaeidi shirin karimi behzad banieghbal nahal mansouri mohammad reza masjedi

chronic granulomatous disease (cgd) is a rare primary immunodeficiency disease. although the most affected patients are diagnosed in childhood, there are several reports of the disease presenting in adult patients. here we present a 40 years old man who was admitted in hospital due to respiratory symptoms and ground glass pattern in high resolution computed tomography of lung. open lung biopsy ...

Journal: :iranian journal of allergy, asthma and immunology 0
shaghayegh tajik immunology, asthma and allergy research institute, tehran university of medical sciences, tehran, iran mohsen badalzadeh immunology, asthma and allergy research institute, tehran university of medical sciences, tehran, iran mohammad reza fazlollahi immunology, asthma and allergy research institute, tehran university of medical sciences, tehran, iran massoud houshmand department of medical genetics, national institute of genetic engineering and biotechnology (nigeb), tehran, iran fariborz zandieh department of asthma, allergy and immunology, bahrami children hospital, tehran university of medical sciences, tehran, iran shamim khandan immunology, asthma and allergy research institute, tehran university of medical sciences, tehran, iran

chronic granulomatous disease (cgd) is a rare primary immunodeficiency disorder due to a genetic defect in one of the components of nicotinamide adenine dinucleotide phosphate (nadph) oxidase complex. this complex is composed of membrane-bound gp91- phox and p22- phox subunits, and cytosolic subunits consisting of p47- phox , p67- phox , and p40- phox . a mutation in cybb gene encoding gp91- ph...

Journal: :The Journal of clinical investigation 1970
R L Baehner D G Nathan M L Karnovsky

Polymorphonuclear leukocytes from patients with chronic granulomatous disease (CGD) exhibit metabolic and bactericidal deficiencies that may be the result of inadequate production of H(2)O(2). A hydrogen peroxide-generating system was, therefore, inserted into CGD leukocytes. This was accomplished by allowing the cells to phagocytize latex spherules coated with glucose oxidase. This produced an...

2016
E. Liana Falcone Jennifer R. Petts Mary Beth Fasano Bradley Ford William M. Nauseef João Farela Neves Maria João Simões Millard L. Tierce M. Teresa de la Morena David E. Greenberg Christa S. Zerbe Adrian M. Zelazny Steven M. Holland

Chronic granulomatous disease (CGD) is a primary immunodeficiency caused by a defect in production of phagocyte-derived reactive oxygen species, which leads to recurrent infections with a characteristic group of pathogens not previously known to include methylotrophs. Methylotrophs are versatile environmental bacteria that can use single-carbon organic compounds as their sole source of energy; ...

2011
Sayandip Mukherjee Giorgia Santilli Michael P. Blundell Susana Navarro Juan A. Bueren Adrian J. Thrasher

Murine models of human genetic disorders provide a valuable tool for investigating the scope for application of induced pluripotent stem cells (iPSC). Here we present a proof-of-concept study to demonstrate generation of iPSC from a mouse model of X-linked chronic granulomatous disease (X-CGD), and their successful differentiation into haematopoietic progenitors of the myeloid lineage. We furth...

2001
Aditya V. Nori Ashok Sreenivas

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Journal: :Proceedings of the National Academy of Sciences of the United States of America 2010
Hidenori Hattori Kulandayan K Subramanian Jiro Sakai Yonghui Jia Yitang Li Timothy F Porter Fabien Loison Bara Sarraj Anongnard Kasorn Hakryul Jo Catlyn Blanchard Dorothy Zirkle Douglas McDonald Sung-Yun Pai Charles N Serhan Hongbo R Luo

Neutrophil chemotaxis plays an essential role in innate immunity, but the underlying cellular mechanism is still not fully characterized. Here, using a small-molecule functional screening, we identified NADPH oxidase-dependent reactive oxygen species as key regulators of neutrophil chemotactic migration. Neutrophils with pharmacologically inhibited oxidase, or isolated from chronic granulomatou...

Journal: :Human mutation 2010
Marcus Gentsch Aneta Kaczmarczyk Karin van Leeuwen Martin de Boer Magdalena Kaus-Drobek Marie-Claire Dagher Petra Kaiser Peter D Arkwright Manfred Gahr Angela Rösen-Wolff Matthias Bochtler Elizabeth Secord Pamela Britto-Williams Gulam Mustafa Saifi Anne Maddalena Ghassan Dbaibo Jacinta Bustamante Jean-Laurent Casanova Dirk Roos Joachim Roesler

Mutations that impair expression or function of the components of the phagocyte NADPH oxidase complex cause chronic granulomatous disease (CGD), which is associated with life-threatening infections and dysregulated granulomatous inflammation. In five CGD patients from four consanguineous families of two different ethnic backgrounds, we found similar genomic homozygous deletions of 1,380 bp comp...

2012
Akira YAMAUCHI Akitoyo ICHINOSE Chikage KAWAI Futoshi KURIBAYASHI Futoshi Kuribayashi

Neutrophils play a crucial role in host defense against microbial infections. During phagocytosis of invading bacteria or fungi, the phagocyte NADPH oxidase produces superoxide. The importance of the oxidase is exemplified by the genetic disorder known as chronic granulomatous disease (CGD). The neutrophils of CGD patients cannot produce superoxide, with the result that affected infants and chi...

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