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http://purl.uniprot.org/citations/21455180http://www.w3.org/1999/02/22-rdf-syntax-ns#typehttp://purl.uniprot.org/core/Journal_Citation
http://purl.uniprot.org/citations/21455180http://www.w3.org/1999/02/22-rdf-syntax-ns#typehttp://purl.uniprot.org/core/Journal_Citation
http://purl.uniprot.org/citations/21455180http://www.w3.org/2000/01/rdf-schema#comment"Cpdm (chronic proliferative dermatitis) mice develop chronic dermatitis and an immunodeficiency with increased serum IgM, symptoms that resemble those of patients with X-linked hyper-IgM syndrome and hypohydrotic ectodermal dysplasia (XHM-ED), which is caused by mutations in NEMO (NF-κB essential modulator; also known as IKBKG). Spontaneous null mutations in the Sharpin (SHANK-associated RH domain interacting protein in postsynaptic density) gene are responsible for the cpdm phenotype in mice. SHARPIN shows significant similarity to HOIL-1L (also known as RBCK1), a component of linear ubiquitin chain assembly complex (LUBAC), which induces NF-κB activation through conjugation of linear polyubiquitin chains to NEMO. Here, we identify SHARPIN as an additional component of LUBAC. SHARPIN-containing complexes can linearly ubiquitinate NEMO and activated NF-κB. Thus, we re-define LUBAC as a complex containing SHARPIN, HOIL-1L, and HOIP (also known as RNF31). Deletion of SHARPIN drastically reduced the amount of LUBAC, which resulted in attenuated TNF-α- and CD40-mediated activation of NF-κB in mouse embryonic fibroblasts (MEFs) or B cells from cpdm mice. Considering the pleomorphic phenotype of cpdm mice, these results confirm the predicted role of LUBAC-mediated linear polyubiquitination in NF-κB activation induced by various stimuli, and strongly suggest the involvement of LUBAC-induced NF-κB activation in various disorders."xsd:string
http://purl.uniprot.org/citations/21455180http://purl.org/dc/terms/identifier"doi:10.1038/nature09815"xsd:string
http://purl.uniprot.org/citations/21455180http://purl.org/dc/terms/identifier"doi:10.1038/nature09815"xsd:string
http://purl.uniprot.org/citations/21455180http://purl.uniprot.org/core/author"Tanaka K."xsd:string
http://purl.uniprot.org/citations/21455180http://purl.uniprot.org/core/author"Tanaka K."xsd:string
http://purl.uniprot.org/citations/21455180http://purl.uniprot.org/core/author"Nakano H."xsd:string
http://purl.uniprot.org/citations/21455180http://purl.uniprot.org/core/author"Nakano H."xsd:string
http://purl.uniprot.org/citations/21455180http://purl.uniprot.org/core/author"Nakagawa T."xsd:string
http://purl.uniprot.org/citations/21455180http://purl.uniprot.org/core/author"Nakagawa T."xsd:string
http://purl.uniprot.org/citations/21455180http://purl.uniprot.org/core/author"Taniguchi M."xsd:string
http://purl.uniprot.org/citations/21455180http://purl.uniprot.org/core/author"Taniguchi M."xsd:string
http://purl.uniprot.org/citations/21455180http://purl.uniprot.org/core/author"Tokunaga F."xsd:string
http://purl.uniprot.org/citations/21455180http://purl.uniprot.org/core/author"Tokunaga F."xsd:string
http://purl.uniprot.org/citations/21455180http://purl.uniprot.org/core/author"Iwai K."xsd:string
http://purl.uniprot.org/citations/21455180http://purl.uniprot.org/core/author"Iwai K."xsd:string
http://purl.uniprot.org/citations/21455180http://purl.uniprot.org/core/author"Nakahara M."xsd:string
http://purl.uniprot.org/citations/21455180http://purl.uniprot.org/core/author"Nakahara M."xsd:string
http://purl.uniprot.org/citations/21455180http://purl.uniprot.org/core/author"Sakata S."xsd:string
http://purl.uniprot.org/citations/21455180http://purl.uniprot.org/core/author"Sakata S."xsd:string
http://purl.uniprot.org/citations/21455180http://purl.uniprot.org/core/author"Saeki Y."xsd:string
http://purl.uniprot.org/citations/21455180http://purl.uniprot.org/core/author"Saeki Y."xsd:string
http://purl.uniprot.org/citations/21455180http://purl.uniprot.org/core/date"2011"xsd:gYear
http://purl.uniprot.org/citations/21455180http://purl.uniprot.org/core/date"2011"xsd:gYear
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