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http://purl.uniprot.org/citations/15578091http://www.w3.org/1999/02/22-rdf-syntax-ns#typehttp://purl.uniprot.org/core/Journal_Citation
http://purl.uniprot.org/citations/15578091http://www.w3.org/2000/01/rdf-schema#comment"Hypomorphic mutations in the zinc finger domain of NF-kappaB essential modulator (NEMO) cause X-linked hyper-IgM syndrome with ectodermal dysplasia (XHM-ED). Here we report that patient B cells are characterized by an absence of Ig somatic hypermutation (SHM) and defective class switch recombination (CSR) despite normal induction of activation-induced cytidine deaminase (AID) and Iepsilon-Cepsilon transcripts. This indicates that AID expression alone is insufficient to support neutralizing antibody responses. Furthermore, we show that patient B cells stimulated with CD40 ligand are impaired in both p65 and c-Rel activation, and whereas addition of IL-4 can enhance p65 activity, c-Rel activity remains deficient. This suggests that these NF-kappaB components have different activation requirements and that IL-4 can augment some but not all NEMO-dependent NF-kappaB signaling. Finally, using microarray analysis of patient B cells we identified downstream effects of impaired NF-kappaB activation and candidate factors that may be necessary for CSR and SHM in B cells."xsd:string
http://purl.uniprot.org/citations/15578091http://purl.org/dc/terms/identifier"doi:10.1172/jci21345"xsd:string
http://purl.uniprot.org/citations/15578091http://purl.uniprot.org/core/author"Liu S."xsd:string
http://purl.uniprot.org/citations/15578091http://purl.uniprot.org/core/author"Jain A."xsd:string
http://purl.uniprot.org/citations/15578091http://purl.uniprot.org/core/author"Means G."xsd:string
http://purl.uniprot.org/citations/15578091http://purl.uniprot.org/core/author"Orange J.S."xsd:string
http://purl.uniprot.org/citations/15578091http://purl.uniprot.org/core/author"Holland S."xsd:string
http://purl.uniprot.org/citations/15578091http://purl.uniprot.org/core/author"Ma C.A."xsd:string
http://purl.uniprot.org/citations/15578091http://purl.uniprot.org/core/author"Lopez-Granados E."xsd:string
http://purl.uniprot.org/citations/15578091http://purl.uniprot.org/core/author"Derry J.M."xsd:string
http://purl.uniprot.org/citations/15578091http://purl.uniprot.org/core/author"Brady W."xsd:string
http://purl.uniprot.org/citations/15578091http://purl.uniprot.org/core/date"2004"xsd:gYear
http://purl.uniprot.org/citations/15578091http://purl.uniprot.org/core/name"J Clin Invest"xsd:string
http://purl.uniprot.org/citations/15578091http://purl.uniprot.org/core/pages"1593-1602"xsd:string
http://purl.uniprot.org/citations/15578091http://purl.uniprot.org/core/title"Specific NEMO mutations impair CD40-mediated c-Rel activation and B cell terminal differentiation."xsd:string
http://purl.uniprot.org/citations/15578091http://purl.uniprot.org/core/volume"114"xsd:string
http://purl.uniprot.org/citations/15578091http://www.w3.org/2004/02/skos/core#exactMatchhttp://purl.uniprot.org/pubmed/15578091
http://purl.uniprot.org/citations/15578091http://xmlns.com/foaf/0.1/primaryTopicOfhttps://pubmed.ncbi.nlm.nih.gov/15578091
http://purl.uniprot.org/uniprot/#_A0A087X1B1-mappedCitation-15578091http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/15578091
http://purl.uniprot.org/uniprot/#_A0A087X1D0-mappedCitation-15578091http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/15578091
http://purl.uniprot.org/uniprot/#_A0A0S2Z349-mappedCitation-15578091http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/15578091
http://purl.uniprot.org/uniprot/#_A0A0S2Z3C7-mappedCitation-15578091http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/15578091
http://purl.uniprot.org/uniprot/#_A0A8V8TPL7-mappedCitation-15578091http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/15578091
http://purl.uniprot.org/uniprot/#_A0A8Q3SI60-mappedCitation-15578091http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/15578091