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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.
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PHAGOCYTES
Essential role of nuclear factor- B for NADPH oxidase activity in normal and anhidrotic ectodermal dysplasia leukocytes
Marcos Luengo-Blanco1,
Carolina Prando1,
Jacinta Bustamante2,
Walmir Cutrim Aragão-Filho3,
Paulo Vitor Soeiro Pereira3,
Jussara Rehder1,
Carolyn Padden4,
Jean-Laurent Casanova2,
Peter E. Newburger4,*, and
Antonio Condino-Neto1,3,*
1 Departments of Pediatrics and Pharmacology, Center for Investigation in Pediatrics, State University of Campinas Medical School, Campinas SP, Brazil;
2 Laboratory of Human Genetics of Infectious Diseases, Inserm, U550, University of Paris René Descartes, Necker Medical School, Paris, France;
3 Department of Immunology, Institute of Biomedical Sciences, University of São Paulo, São Paulo, Brazil; and
4 Departments of Pediatrics and Cancer Biology, University of Massachusetts Medical School, Worcester
This work investigated the functional role of nuclear factor– B (NF-B) 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-B (IB -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-B 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 (X910 CGD). NCF1 gene expression in EDA-ID S32I cells was decreased compared with healthy control cells and similar to that in 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.
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