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Phagocytic Cell Disorders – Primary Immunodeficiency

by Peter Delves, PhD
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    There are a number of gene defects that can affect phagocytic cells, and these are listed here. Defective genes for components of the NADPH oxidase can result in chronic granulomatous disease. And here we see a number of typical infections, with an increased instance and severity of disease with Staph aureus, Aspergillus fumigatus, Candida albicans and so on. A defect in the CD18 β-subunit; CD18 is an integrin adhesion molecule, leads to leukocyte adhesion deficiency type I. We see an increased instance of pyogenic bacteria. In contrast, a defective gene for the GDP-fucose transporter leads to leukocyte adhesion deficiency or LAD II; again, with an increased incidence of pyogenic bacteria. Kindlin 3 deficiency leads to leukocyte adhesion deficiency type III, again with an increased incidence of pyogenic bacteria. And then a defect in the LYST gene leads to the disorder called Chediak-Higashi syndrome. And a range of Staphylococci and Streptococci, and other species are seen with an increased incidence in this condition. Let’s have a look in a little bit more detail at chronic granulomatous disease. In the vast majority of patients, this is due to a defect in subunits of the nicotinamide adenine dinucleotide phosphate oxidase, the NADPH oxidase. It affects monocytes, macrophages and neutrophils. And they fail to produce reactive oxygen intermediates that are required to kill engulfed microorganisms. The NADPH oxidase consists of a number of different subunits as you can see here. The function of this oxidase is to produce reactive oxygen species that are involved in killing engulfed microorganisms. A defect in the gene encoding the gp91-phox component of the NADPH oxidase, is the X-linked form of this disease because that gene is present on the X chromosome. The genes for the other components of the NADPH oxidase are found on the non-sex chromosomes, in...

    About the Lecture

    The lecture Phagocytic Cell Disorders – Primary Immunodeficiency by Peter Delves, PhD is from the course Immunodeficiency and Immune Deficiency Diseases. It contains the following chapters:

    • Defects of Phagocytic Cells
    • Chronic Granulomatous Disease
    • Leukocyte Adhesion Deficiency
    • Chediak-Higashi Syndrome

    Included Quiz Questions

    1. gp91phox
    2. p22phox
    3. CD18 β-subunit
    4. LYST
    5. Myeloperoxidase
    1. Chediak-Higashi Syndrome
    2. Leukocyte Adhesion Deficiency II
    3. Chronic Granulomatous Disease
    4. G6PD Deficiency
    5. Leukocyte Adhesion Deficiency III
    1. A mutation in kindlin 3
    2. A lack of CD18 β-subunit
    3. A defective GDP-fucose transporter
    4. Inability to fucosylate sialyl Lewis x structures
    5. A defect in NADPH oxidase
    1. Neutrophils, NK cells, and Cytotoxic T cells
    2. Neutrophils, Basophils, and Cytotoxic T cells
    3. NK cells, Cytotoxic T cells, and Basophils
    4. Neutrophils, NK cells, and Basophils
    5. NK cells, Neutrophils, and Helper T cells

    Author of lecture Phagocytic Cell Disorders – Primary Immunodeficiency

     Peter Delves, PhD

    Peter Delves, PhD


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