O papel do fator nuclear Kappa B (NF-k B) na expressão do gene CYBB do sistema NADPH oxidase humano / The role of nuclear factor k-B (NF-kB) CYBB gene expression activity of the human NADPH oxidase system

AUTOR(ES)
DATA DE PUBLICAÇÃO

2008

RESUMO

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 gene CYBB. U937 cells with a stably transfected repressor of NF-?B (I-?Ba-S32A/ S36A) demonstrated significantly lower superoxide release and lower CYBB 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 (EDAID), an inherited disorder of NF-?B function (DAE-IDI?B S32I e DAE-ID NEMO/IKK? X420W). 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° CGD). Gel shift assays demonstrated loss of recombinant human p50 binding to a NF-?B site 5 to the CYBB gene inU937 cells treated with NF-?B inhibitors, repressor-transfected U937 cells, and EDA-ID patients cells. These studies show that NF-?B is necessary for CYBB gene expression and activation of the phagocyte NADPH oxidase in this model system

ASSUNTO(S)

superoxide doença granuomatosa cronica expressão genica nadph oxidase nadph oxidase - análise superoxido gene expression chronic granulomatosis disease

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