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Blood, 15 August 2008, Vol. 112, No. 4, pp. 1453-1460. Prepublished online as a Blood First Edition Paper on June 3, 2008; DOI 10.1182/blood-2007-07-099267. This Article Full Text (PDF) Supplemental Figures All Versions of this Article: blood-2007-07-099267v1 112/4/1453    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Luengo-Blanco, M. Articles by Condino-Neto, A. Search for Related Content PubMed PubMed Citation Articles by Luengo-Blanco, M. Articles by Condino-Neto, A. Related Collections Related Articles in Blood Online Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted July 3, 2007 Accepted April 1, 2008 Essential role of nuclear factor-kappa B for NADPH oxidase activity in normal and anhidrotic ectodermal dysplasia leukocytes Marcos Luengo-Blanco, Carolina Prando, Jacinta Bustamante, Walmir Cutrim Aragao-Filho, Paulo Vitor Soeiro Pereira, Jussara Rehder, Carolyn Padden, Jean-Laurent Casanova, Peter E. Newburger*, and Antonio Condino-Neto Department of Pediatrics, State University of Campinas, Campinas, SP, Brazil Laboratory of Human Genetics of Infectious Diseases, Necker Medical School, Paris, France Department of Immunology, Institute of Biomedical Sciences, University of Sao Paulo, Sao Paulo, SP, Brazil Department of Pediatrics, University of Massachusetts Medical School, Worcester, MA, United States * Corresponding author; email: peter.newburger{at}umassmed.edu. This work investigated the functional role of nuclear factor-kappa B (NF-B) in respiratory burst activity and in expression of the human phagocyte NADPH oxidase genes CYBB, CYBA, NCF1, and NCF2. U937 cells with a stably transfected repressor of NF-B (I-B-S32A/S36A) demonstrated significantly lower superoxide release and lower CYBB and NCF1 gene expression compared to control U937 cells. We further tested EBV-transformed B cells from patients with anhidrotic ectodermal dysplasia with immunodeficiency (EDA-ID), an inherited disorder of NF-B function. Superoxide release and CYBB gene expression by EDA-ID cells were significantly decreased compared to normal cells and similar to X-linked chronic granulomatous disease (X910 CGD) patient cells. NCF1 gene expression in EDA-ID S32I cells was decreased compared to normal control cells and similar to autosomal recessive (A470) CGD cells. Gel shift assays demonstrated loss of recombinant human p50 binding to a NF-B site 5' to the CYBB gene in U937 cells treated with NF-B inhibitors, repressor-transfected U937 cells, and EDA-ID patients' cells. Zymosan phagocytosis was not affected by transfection of U937 cells with the NF-B repressor. These studies show that NF-B is necessary for CYBB and NCF1 gene expression and activation of the phagocyte NADPH oxidase in this model system. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? Related Articles in Blood Online: Response: Homing defect in hematopoietic cells from Fanconi anemia patients Xiaoling Zhang and Qishen Pang Blood 2009 113: 5362. [Full Text] [PDF] Nuclear factor-B is not essential for NADPH oxidase activity in neutrophils from anhidrotic ectodermal dysplasia patients Bram J. van Raam, Robin van Bruggen, Anton T. J. Tool, Machiel H. Jansen, Adilia Warris, Stephen Jolles, and Taco W. Kuijpers Blood 2009 113: 5362-5363. [Full Text] [PDF] This article has been cited by other articles: B. J. van Raam, R. van Bruggen, A. T. J. Tool, M. H. Jansen, A. Warris, S. Jolles, and T. W. Kuijpers Nuclear factor-{kappa}B is not essential for NADPH oxidase activity in neutrophils from anhidrotic ectodermal dysplasia patients Blood, May 21, 2009; 113(21): 5362 - 5363. [Full Text] [PDF]

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