LymphedemaV1, France, Analysis, bibRecord, 000424

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

Identifieur interne : 000424 ( France/Analysis ); précédent : 000423; suivant : 000425

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

Auteurs : Marcos Luengo-Blanco [Brésil] ; Carolina Prando [Brésil] ; Jacinta Bustamante [France] ; Walmir Cutrim Aragão-Filho [Brésil] ; Paulo Vitor Soeiro Pereira [Brésil] ; Jussara Rehder [Brésil] ; Carolyn Padden ; Jean-Laurent Casanova [France] ; Peter E. Newburger ; Antonio Condino-Neto [Brésil]

Source :

Blood [ 0006-4971 ] ; 2008.

RBID : PMC:2515116

Abstract

This work investigated the functional role of nuclear factor–κB (NF-κB) 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-κB (IκBα-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-κB 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-κB site 5′ to the CYBB gene in U937 cells treated with NF-κB inhibitors, repressor-transfected U937 cells, and EDA-ID patients' cells. Zymosan phagocytosis was not affected by transfection of U937 cells with the NF-κB repressor. These studies show that NF-κB is necessary for CYBB and NCF1 gene expression and activation of the phagocyte NADPH oxidase in this model system.

Url:

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2515116

DOI: 10.1182/blood-2007-07-099267
PubMed: 18523147
PubMed Central: 2515116

Affiliations:

Links to Exploration step

PMC:2515116

Le document en format XML

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<author><name sortKey="Rehder, Jussara" sort="Rehder, Jussara" uniqKey="Rehder J" first="Jussara" last="Rehder">Jussara Rehder</name>
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<front><div type="abstract" xml:lang="en"><p>This work investigated the functional role of nuclear factor–κB (NF-κB) in respiratory burst activity and in expression of the human phagocyte nicotinamide adenine dinucleotide phosphate (NADPH) oxidase genes <italic>CYBB</italic>
, <italic>CYBA</italic>
, <italic>NCF1</italic>
, and <italic>NCF2</italic>
. U937 cells with a stably transfected repressor of NF-κB (IκBα-S32A/S36A) demonstrated significantly lower superoxide release and lower <italic>CYBB</italic>
 and <italic>NCF1</italic>
 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-κB function. Superoxide release and <italic>CYBB</italic>
 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<sup>0</sup>
 CGD). <italic>NCF1</italic>
 gene expression in EDA-ID S32I cells was decreased compared with healthy control cells and similar to that in autosomal recessive (A47<sup>0</sup>
) CGD cells. Gel shift assays demonstrated loss of recombinant human p50 binding to a NF-κB site 5′ to the <italic>CYBB</italic>
 gene in U937 cells treated with NF-κB inhibitors, repressor-transfected U937 cells, and EDA-ID patients' cells. Zymosan phagocytosis was not affected by transfection of U937 cells with the NF-κB repressor. These studies show that NF-κB is necessary for <italic>CYBB</italic>
 and <italic>NCF1</italic>
 gene expression and activation of the phagocyte NADPH oxidase in this model system.</p>
</div>
</front>
</TEI>
<affiliations><list><country><li>Brésil</li>
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</orgName>
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<tree><noCountry><name sortKey="Newburger, Peter E" sort="Newburger, Peter E" uniqKey="Newburger P" first="Peter E." last="Newburger">Peter E. Newburger</name>
<name sortKey="Padden, Carolyn" sort="Padden, Carolyn" uniqKey="Padden C" first="Carolyn" last="Padden">Carolyn Padden</name>
</noCountry>
<country name="Brésil"><region name="État de São Paulo"><name sortKey="Luengo Blanco, Marcos" sort="Luengo Blanco, Marcos" uniqKey="Luengo Blanco M" first="Marcos" last="Luengo-Blanco">Marcos Luengo-Blanco</name>
</region>
<name sortKey="Aragao Filho, Walmir Cutrim" sort="Aragao Filho, Walmir Cutrim" uniqKey="Aragao Filho W" first="Walmir Cutrim" last="Aragão-Filho">Walmir Cutrim Aragão-Filho</name>
<name sortKey="Condino Neto, Antonio" sort="Condino Neto, Antonio" uniqKey="Condino Neto A" first="Antonio" last="Condino-Neto">Antonio Condino-Neto</name>
<name sortKey="Condino Neto, Antonio" sort="Condino Neto, Antonio" uniqKey="Condino Neto A" first="Antonio" last="Condino-Neto">Antonio Condino-Neto</name>
<name sortKey="Pereira, Paulo Vitor Soeiro" sort="Pereira, Paulo Vitor Soeiro" uniqKey="Pereira P" first="Paulo Vitor Soeiro" last="Pereira">Paulo Vitor Soeiro Pereira</name>
<name sortKey="Prando, Carolina" sort="Prando, Carolina" uniqKey="Prando C" first="Carolina" last="Prando">Carolina Prando</name>
<name sortKey="Rehder, Jussara" sort="Rehder, Jussara" uniqKey="Rehder J" first="Jussara" last="Rehder">Jussara Rehder</name>
</country>
<country name="France"><region name="Île-de-France"><name sortKey="Bustamante, Jacinta" sort="Bustamante, Jacinta" uniqKey="Bustamante J" first="Jacinta" last="Bustamante">Jacinta Bustamante</name>
</region>
<name sortKey="Casanova, Jean Laurent" sort="Casanova, Jean Laurent" uniqKey="Casanova J" first="Jean-Laurent" last="Casanova">Jean-Laurent Casanova</name>
</country>
</tree>
</affiliations>
</record>

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

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

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Abstract

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