Essential role of nuclear factor-kappa B for NADPH oxidase activity in normal and anhildrotic ectodermal dysplasia leukocytes

Essential role of nuclear factor-kappa B for NADPH oxidase activity in normal and anhildrotic ectodermal dysplasia leukocytes

Autor Luengo-Blanco, Marcos Autor UNIFESP Google Scholar
Prando, Carolina Google Scholar
Bustamante, Jacinta Google Scholar
Arago-Filho, Walmir Cutrim Google Scholar
Pereira, Paulo Vitor Soeiro Google Scholar
Rehder, Jussara Google Scholar
Padden, Carolyn Google Scholar
Casanova, Jean-Laurent Google Scholar
Newburger, Peter E. Google Scholar
Condino-Neto, Antonio Google Scholar
Instituição Univ Massachusetts
Universidade Federal de São Paulo (UNIFESP)
Universidade Estadual de Campinas (UNICAMP)
Univ Paris 05
Universidade de São Paulo (USP)
Resumo This work investigated the functional role of nuclear factor-kappa B (NF-kappa 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-kappa B (IKB alpha-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 disorderof NF-kappa 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 (X91 degrees CGD). NCF1 gene expression in EDA-ID S321 cells was decreased compared with healthy control cells and similar to that in autosomal recessive (A47 degrees) CGD cells. Gel shift assays demonstrated loss of recombinant human p50 binding to a NF-kappa B site 5' to the CYBB gene in U937 cells treated with NF-kappa B inhibitors, repressor-transfected U937 cells, and EDA-ID patients cells. Zymosan phagocytosis was not affected by transfection of U937 cells with the NF-kappa B repressor. These studies show that NF-kappa B is necessary for CYBB and NCF1 gene expression and activation of the phagocyte NADPH oxidase in this model system.
Idioma Inglês
Data 2008-08-15
Publicado em Blood. Washington: Amer Soc Hematology, v. 112, n. 4, p. 1453-1460, 2008.
ISSN 0006-4971 (Sherpa/Romeo, fator de impacto)
Editor Amer Soc Hematology
Extensão 1453-1460
Direito de acesso Acesso aberto Open Access
Tipo Artigo
Web of Science WOS:000258392300072

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