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    Essential role of nuclear factor-kappaB for NADPH oxidase activity in normal and anhidrotic ectodermal dysplasia leukocytes

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    Authors
    Luengo-Blanco, Marcos
    Prando, Carolina
    Bustamante, Jacinta
    Aragao-Filho, Walmir Cutrim
    pereira, Paulo Vitor Soeiro
    Rehder, Jussara
    Padden, Carolyn
    Casanova, Jean-Laurent
    Newburger, Peter E.
    Condino-Neto, Antonio
    UMass Chan Affiliations
    Department of Pediatrics
    Document Type
    Journal Article
    Publication Date
    2008-08-15
    Keywords
    Cell Line, Transformed
    Cells, Cultured
    Ectodermal Dysplasia
    Gene Expression
    Granulomatous Disease, Chronic
    Humans
    Leukocytes
    Membrane Glycoproteins
    NADPH Oxidase
    NF-kappa B
    Phagocytes
    Phagocytosis
    Hematology
    Oncology
    Pediatrics
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    Link to Full Text
    http://dx.doi.org/10.1182/blood-2007-07-099267
    Abstract
    This work investigated the functional role of nuclear factor-kappaB (NF-kappaB) in respiratory burst activity and in expression of the human phagocyte nicotinamide adenine dinucleotide phosphate (NADPH) oxidase genes CYBB, CYBA, NCF1, and NCF2. U937 cells with a stably transfected repressor of NF-kappaB (IkappaBalpha-S32A/S36A) demonstrated significantly lower superoxide release and lower CYBB and NCF1 gene expression compared with control U937 cells. We further tested Epstein-Barr virus (EBV)-transformed B cells from patients with anhidrotic ectodermal dysplasia with immunodeficiency (EDA-ID), an inherited disorder of NF-kappaB function. Superoxide release and CYBB gene expression by EDA-ID cells were significantly decreased compared with healthy cells and similar to cells from patients with X-linked chronic granulomatous disease (X91(0) CGD). NCF1 gene expression in EDA-ID S32I cells was decreased compared with healthy control cells and similar to that in autosomal recessive (A47(0)) CGD cells. Gel shift assays demonstrated loss of recombinant human p50 binding to a NF-kappaB site 5' to the CYBB gene in U937 cells treated with NF-kappaB inhibitors, repressor-transfected U937 cells, and EDA-ID patients' cells. Zymosan phagocytosis was not affected by transfection of U937 cells with the NF-kappaB repressor. These studies show that NF-kappaB is necessary for CYBB and NCF1 gene expression and activation of the phagocyte NADPH oxidase in this model system.
    Source
    Blood. 2008 Aug 15;112(4):1453-60. Epub 2008 Jun 3. doi 10.1182/blood-2007-07-099267. Link to article on publisher's website
    DOI
    10.1182/blood-2007-07-099267
    Permanent Link to this Item
    http://hdl.handle.net/20.500.14038/43309
    PubMed ID
    18523147
    Related Resources
    Link to article in PubMed
    ae974a485f413a2113503eed53cd6c53
    10.1182/blood-2007-07-099267
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