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Defective lymphoid organogenesis underlies the immune deficiency caused by a heterozygous S32I mutation in IκBα
Patients with ectodermal dysplasia with immunodeficiency (ED-ID) caused by mutations in the inhibitor of NF-κB α (IκBα) are susceptible to severe recurrent infections, despite normal T and B cell numbers and intact in vitro lymphocyte function. Moreover, the outcome of hematopoietic stem cell transp...
Autores principales: | Mooster, Jana L., Le Bras, Severine, Massaad, Michel J., Jabara, Haifa, Yoon, Juhan, Galand, Claire, Heesters, Balthasar A., Burton, Oliver T., Mattoo, Hamid, Manis, John, Geha, Raif S. |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
The Rockefeller University Press
2015
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4322042/ https://www.ncbi.nlm.nih.gov/pubmed/25601653 http://dx.doi.org/10.1084/jem.20140979 |
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