This is a group of disorders inherited as Chronic granulomatous disease (CGD) either an X-linked or autosomal recessive gene that affects neutrophil microbicidal function. This disease accounts for 70 percent of X-linked disease the cases, and it tends to be more severe. Symptoms of CGD include recurrent Suppurative infections, pneumonia, osteomyelitis, what? draining adenopathy, liver abscesses, dermatitis, and hypergammaglobulinemia. CGD is the most common and best The neutrophil abnormalities characterized of this type of abnormalities? There are three different autosomal Nicotinamide adenine dinucleotide phosphate recessive genes involved, and all of these (NADPH) oxidase. affect subunits of what? What normally leads to the production of Normally, neutrophil stimulation reactive oxygen molecules, such as hydrogen peroxide (H2O2), by NADPH oxidase on the plasma membrane? These granules fuse with, and release Neutrophil granules their contents into, the forming phagosome, as organisms are phagocytized. What is used by the granule enzyme Hydrogen peroxide myeloperoxidase to generate the potent microbicidal hypochlorous acid. The process of generating partially An increase in oxygen consumption. reduced forms of oxygen by stimulated neutrophils was first detected as what? This response was originally burst.” “oxidative termed the neutrophil burst”; “respiratory a more correct term is called? In CGD, neutrophils are unable to do undergo the oxidative burst what? CGD was historically diagnosed by The dye nitro-blue tetrazolium (NBT) measuring the ability of a patient’s neutrophils to reduce what dye? More recently, this has become a flow cytometric assay available. In this assay, what are neutrophils dihydrorhodamine (DHR) labeled with? DHR will do what when it is reduced. fluoresce The neutrophils are then activated Phorbol myristate acetate (PMA) using this, which is mitogenic for neutrophils.
This is a group of disorders inherited as either an X-linked or autosomal recessive gene that affects neutrophil microbicidal function. Chronic granulomatous disease (CGD) This disease accounts for 70 percent of the cases, and it tends to be more severe. X-linked disease Symptoms of CGD include recurrent what? Suppurative infections, pneumonia, osteomyelitis, draining adenopathy, liver abscesses, dermatitis, and hypergammaglobulinemia. CGD is the most common and best characterized of this type of abnormalities? The neutrophil abnormalities There are three different autosomal recessive genes involved, and all of these affect subunits of what? Nicotinamide adenine dinucleotide phosphate (NADPH) oxidase. What normally leads to the production of reactive oxygen molecules, such as hydrogen peroxide (H2O2), by NADPH oxidase on the plasma membrane? Normally, neutrophil stimulation These granules fuse with, and release their contents into, the forming phagosome, as organisms are phagocytized. Neutrophil granules What is used by the granule enzyme myeloperoxidase to generate the potent microbicidal hypochlorous acid. Hydrogen peroxide The process of generating partially reduced forms of oxygen by stimulated neutrophils was first detected as what? An increase in oxygen consumption. This response was originally termed the neutrophil “respiratory burst”; a more correct term is called? “oxidative burst.” In CGD, neutrophils are unable to do what? undergo the oxidative burst CGD was historically diagnosed by measuring the ability of a patient’s neutrophils to reduce what dye? The dye nitro-blue tetrazolium (NBT) More recently, this has become available. a flow cytometric assay In this assay, what are neutrophils labeled with? dihydrorhodamine (DHR) DHR will do what when it is reduced. fluoresce The neutrophils are then activated using this, which is mitogenic for neutrophils. Phorbol myristate acetate (PMA)
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