Essential role of nuclear factor-κB for NADPH oxidase activity in normal and anhidrotic ectodermal dysplasia leukocytes
Identifieur interne : 002D54 ( Ncbi/Merge ); précédent : 002D53; suivant : 002D55Essential 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.
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
Url:
DOI: 10.1182/blood-2007-07-099267
PubMed: 18523147
PubMed Central: 2515116
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<author><name sortKey="Padden, Carolyn" sort="Padden, Carolyn" uniqKey="Padden C" first="Carolyn" last="Padden">Carolyn Padden</name>
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<author><name sortKey="Casanova, Jean Laurent" sort="Casanova, Jean Laurent" uniqKey="Casanova J" first="Jean-Laurent" last="Casanova">Jean-Laurent Casanova</name>
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<wicri:noCountry code="subfield">Worcester</wicri:noCountry>
</affiliation>
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<author><name sortKey="Condino Neto, Antonio" sort="Condino Neto, Antonio" uniqKey="Condino Neto A" first="Antonio" last="Condino-Neto">Antonio Condino-Neto</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>
<pmc article-type="research-article" xml:lang="EN"><pmc-comment>The publisher of this article does not allow downloading of the full text in XML form.</pmc-comment>
<front><journal-meta><journal-id journal-id-type="nlm-ta">Blood</journal-id>
<journal-id journal-id-type="hwp">bloodjournal</journal-id>
<journal-id journal-id-type="pmc">blood</journal-id>
<journal-id journal-id-type="publisher-id">Blood</journal-id>
<journal-title>Blood</journal-title>
<issn pub-type="ppub">0006-4971</issn>
<issn pub-type="epub">1528-0020</issn>
<publisher><publisher-name>American Society of Hematology</publisher-name>
<publisher-loc>Washington, DC</publisher-loc>
</publisher>
</journal-meta>
<article-meta><article-id pub-id-type="pmid">18523147</article-id>
<article-id pub-id-type="pmc">2515116</article-id>
<article-id pub-id-type="publisher-id">2007/099267</article-id>
<article-id pub-id-type="doi">10.1182/blood-2007-07-099267</article-id>
<article-categories><subj-group subj-group-type="heading"><subject>Phagocytes</subject>
</subj-group>
<series-title>Phagocytes</series-title>
</article-categories>
<title-group><article-title>Essential role of nuclear factor-κB for NADPH oxidase activity in normal and anhidrotic ectodermal dysplasia leukocytes</article-title>
</title-group>
<contrib-group><contrib contrib-type="author"><name><surname>Luengo-Blanco</surname>
<given-names>Marcos</given-names>
</name>
<xref ref-type="aff" rid="aff1">1</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Prando</surname>
<given-names>Carolina</given-names>
</name>
<xref ref-type="aff" rid="aff1">1</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Bustamante</surname>
<given-names>Jacinta</given-names>
</name>
<xref ref-type="aff" rid="aff2">2</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Aragão-Filho</surname>
<given-names>Walmir Cutrim</given-names>
</name>
<xref ref-type="aff" rid="aff3">3</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Pereira</surname>
<given-names>Paulo Vitor Soeiro</given-names>
</name>
<xref ref-type="aff" rid="aff3">3</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Rehder</surname>
<given-names>Jussara</given-names>
</name>
<xref ref-type="aff" rid="aff1">1</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Padden</surname>
<given-names>Carolyn</given-names>
</name>
<xref ref-type="aff" rid="aff4">4</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Casanova</surname>
<given-names>Jean-Laurent</given-names>
</name>
<xref ref-type="aff" rid="aff2">2</xref>
</contrib>
<contrib contrib-type="author" corresp="yes"><name><surname>Newburger</surname>
<given-names>Peter E.</given-names>
</name>
<xref ref-type="aff" rid="aff4">4</xref>
<xref ref-type="author-notes" rid="FN1">*</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Condino-Neto</surname>
<given-names>Antonio</given-names>
</name>
<xref ref-type="aff" rid="aff1">1</xref>
<xref ref-type="aff" rid="aff3">3</xref>
<xref ref-type="author-notes" rid="FN1">*</xref>
</contrib>
<aff id="aff1"><label>1</label>
Departments of Pediatrics and Pharmacology, Center for Investigation in Pediatrics, State University of Campinas Medical School, Campinas SP, Brazil;</aff>
<aff id="aff2"><label>2</label>
Laboratory of Human Genetics of Infectious Diseases, Inserm, U550, University of Paris René Descartes, Necker Medical School, Paris, France;</aff>
<aff id="aff3"><label>3</label>
Department of Immunology, Institute of Biomedical Sciences, University of São Paulo, São Paulo, Brazil; and</aff>
<aff id="aff4"><label>4</label>
Departments of Pediatrics and Cancer Biology, University of Massachusetts Medical School, Worcester</aff>
</contrib-group>
<author-notes><fn fn-type="equal" id="FN1"><label>*</label>
<p>P.E.N. and A.C.-N. contributed equally to the development, execution, and interpretation of this work.</p>
</fn>
</author-notes>
<pub-date pub-type="ppub"><day>15</day>
<month>8</month>
<year>2008</year>
</pub-date>
<pub-date pub-type="epreprint"><day>3</day>
<month>6</month>
<year>2008</year>
</pub-date>
<pub-date pub-type="pmc-release"><day>15</day>
<month>8</month>
<year>2009</year>
</pub-date>
<pmc-comment> PMC Release delay is 12 months and 0 days and was based on the
. </pmc-comment>
<volume>112</volume>
<issue>4</issue>
<fpage>1453</fpage>
<lpage>1460</lpage>
<history><date date-type="received"><day>2</day>
<month>7</month>
<year>2007</year>
</date>
<date date-type="accepted"><day>1</day>
<month>4</month>
<year>2008</year>
</date>
</history>
<copyright-statement>© 2008 by The American Society of Hematology</copyright-statement>
<copyright-year>2008</copyright-year>
<self-uri xlink:title="pdf" xlink:type="simple" xlink:href="zh801608001453.pdf"></self-uri>
<abstract><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>
</abstract>
<contract-num rid="CS100">DK54369</contract-num>
<contract-sponsor id="CS100" xlink:href="http://www.nih.gov">National Institutes of Health</contract-sponsor>
</article-meta>
</front>
</pmc>
<affiliations><list><country><li>Brésil</li>
<li>France</li>
</country>
<region><li>État de São Paulo</li>
<li>Île-de-France</li>
</region>
<settlement><li>Paris</li>
<li>São Paulo</li>
</settlement>
<orgName><li>Université de São Paulo</li>
</orgName>
</list>
<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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