نتایج جستجو برای: chronic granulomatous disease cgd

تعداد نتایج: 1773487  

Journal: :The Journal of clinical investigation 1980
P E Newburger M S Kruskall J M Rappeport S H Robinson M E Chovaniec H J Cohen

Chronic granulomatous disease (CGD), an often fatal syndrome of recurrent infections results from the inability of patients' peripheral blood phagocytic leukocytes to generate superoxide despite otherwise normal phagocytic functions such as ingestion and degranulation. Circulating granulocytes and monocytes are the progeny of bone marrow progenitor cells, colony-forming units in culture. We com...

Journal: :Saudi medical journal 2006
Maryam Ayatollahi Bita Geramizadeh

OBJECTIVE To develop an application that is simple and reliable using the nitroblue tetrazolium (NBT) method that clearly differentiates chronic granulomatous disease (CGD) patients with heterozygous carriers in groups suspected with CGD. METHODS This study was carried out in Shiraz University of Medical Sciences from October 2002 and March 2004. The study included 260 samples consisting of 1...

2012
Maria J. Gutierrez George D. McSherry Faoud T. Ishmael Alexandra A. Horwitz Gustavo Nino

Chronic granulomatous disease (CGD) is characterized by inherited immune defects resulting from mutations in the NADPH oxidase complex genes. The X-linked type of CGD is caused by defects in the CYBB gene that encodes gp91-phox, a fundamental component of the NADPH oxidase complex. This mutation originates the most common and severe form of CGD, which typically has absence of NADPH oxidase func...

Journal: :Clinical and experimental immunology 1986
C J Heijnen J W van der Meer B J Zegers

Phagocytic cells of patients with chronic granulomatous disease (CGD) are severely impaired in the killing process, on the basis of defective oxygen metabolism. In this study we investigated the antigen-presenting function of monocytes (i.e. adherent cells) of CGD patients. Adherent cells of CGD patients were investigated for their capacity to present ovalbumin (OA) in such a way that T helper ...

Journal: :Blood 2012
Susan Moir Suk See De Ravin Brian H Santich Jin Young Kim Jacqueline G Posada Jason Ho Clarisa M Buckner Wei Wang Lela Kardava Mary Garofalo Beatriz E Marciano Jody Manischewitz Lisa R King Surender Khurana Tae-Wook Chun Hana Golding Anthony S Fauci Harry L Malech

CD27(+) memory B cells are reduced in the blood of patients with chronic granulomatous disease (CGD) for reasons and consequences that remain unclear. Here we confirm not only decreased CD27(+) but also IgG(+) B cells in the blood of CGD patients compared with healthy donors (HDs). However, among IgG(+) B cells, the ratio of CD27(-) to CD27(+) was significantly higher in CGD patients compared w...

Journal: :The Biochemical journal 1996
C D Porter F Kuribayashi M H Parkar D Roos C Kinnon

NADPH oxidase cytochrome b558 consists of two subunits, gp91-phox and p22-phox, defects of which result in chronic granulomatous disease (CGD). The nature of the interaction between these subunits has yet to be determined. Absence of p22-phox in autosomal CGD patient-derived B-cell lines results in detectable levels of an incompletely glycosylated gp91-phox precursor. We have detected this same...

Journal: :Blood 2000
J Roesler J T Curnutte J Rae D Barrett P Patino S J Chanock A Goerlach

Chronic granulomatous disease (CGD) is an inherited disease caused by defects in the superoxide-generating nicotinamide adenine dinucleotide phosphate (NADPH) oxidase of phagocytes. Genetic lesions in any of 4 components of this antimicrobial enzyme have been detected. Family-specific mutations are found in 3 of 4 forms of CGD due to deficiencies of the gp91-phox, p22-phox, and p67-phox genes. ...

2016
E. Liana Falcone Loreto Abusleme Muthulekha Swamydas Michail S. Lionakis Li Ding Amy P. Hsu Adrian M. Zelazny Niki M. Moutsopoulos Douglas B. Kuhns Clay Deming Mariam Quiñones Julia A. Segre Clare E. Bryant Steven M. Holland

Background: Chronic granulomatous disease (CGD) is caused by defects in nicotinamide adenine dinucleotide phosphate oxidase 2 (NOX2) complex subunits (gp91 (a.k.a. Nox2), p47, p67, p22, p40) leading to reduced phagocyte-derived reactive oxygen species production. Almost half of patients with CGD develop inflammatory bowel disease, and the involvement of the intestinal microbiome in relation to ...

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