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dc.contributor.authorFuente García, Miguel Ángel de la es
dc.contributor.authorSasahara, Yoji
dc.contributor.authorCalamito, Marco
dc.contributor.authorAntón, Inés María
dc.contributor.authorElkhal, Abdallah
dc.contributor.authorGallego, María Dolores
dc.contributor.authorSuresh, Koduro
dc.contributor.authorSiminovitch, Katherine
dc.contributor.authorOchs, Hans D.
dc.contributor.authorAnderson, Kenneth C.
dc.contributor.authorRosen, Fred S.
dc.contributor.authorGeha, Raif S.
dc.contributor.authorRamesh, Narayanaswamy
dc.date.accessioned2015-03-23T12:36:37Z
dc.date.available2015-03-23T12:36:37Z
dc.date.issued2007
dc.identifier.citationProceedings of the National Academy of Sciences U S A. 2007 Jan 16;104(3):926-31es
dc.identifier.issn0027-8424es
dc.identifier.urihttp://uvadoc.uva.es/handle/10324/9826
dc.descriptionProducción Científicaes
dc.description.abstractWiskott–Aldrich syndrome protein (WASP) is in a complex with WASP-interacting protein (WIP). WASP levels, but not mRNA levels, were severely diminished in T cells from WIP / mice and were increased by introduction of WIP in these cells. The WASP binding domain of WIP was shown to protect WASP from degradation by calpain in vitro. Treatment with the proteasome inhibitors MG132 and bortezomib increased WASP levels in T cells from WIP / mice and in T and B lymphocytes from two WAS patients with missense mutations (R86H and T45M) that disrupt WIP binding. The calpain inhibitor calpeptin increased WASP levels in activated T and B cells from the WASP patients, but not in primary T cells from the patients or from WIP / mice. Despite its ability to increase WASP levels proteasome inhibition did not correct the impaired IL-2 gene expression and low F-actin content in T cells from the R86H WAS patient. These results demonstrate that WIP stabilizes WASP and suggest that it may also be important for its functiones
dc.format.mimetypeapplication/pdfes
dc.language.isoenges
dc.publisherNational Academy of Scienceses
dc.rights.accessRightsinfo:eu-repo/semantics/openAccesses
dc.rights.urihttp://creativecommons.org/licenses/by-nc-nd/4.0/
dc.subjectWiskott Aldrich, Síndrome protéicoes
dc.titleWIP is a chaperone for Wiskott–Aldrich syndrome protein (WASP)es
dc.typeinfo:eu-repo/semantics/articlees
dc.identifier.doi10.1073 pnas.0610275104es
dc.identifier.publicationfirstpage926es
dc.identifier.publicationissue3es
dc.identifier.publicationlastpage931es
dc.identifier.publicationtitleProceedings of the National Academy of Scienceses
dc.identifier.publicationvolume104es
dc.peerreviewedSIes
dc.rightsAttribution-NonCommercial-NoDerivatives 4.0 International


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