Expansion of immunoglobulin-secreting cells and defects in B cell tolerance in Rag-dependent immunodeficiency

The contribution of B cells to the pathology of Omenn syndrome and leaky severe combined immunodeficiency (SCID) has not been previously investigated. We have studied a mut/mut mouse model of leaky SCID with a homozygous Rag1 S723C mutation that impairs, but does not abrogate, V(D)J recombination ac...

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Detalles Bibliográficos
Autores principales: Walter, Jolan E., Rucci, Francesca, Patrizi, Laura, Recher, Mike, Regenass, Stephan, Paganini, Tiziana, Keszei, Marton, Pessach, Itai, Lang, Philipp A., Poliani, Pietro Luigi, Giliani, Silvia, Al-Herz, Waleed, Cowan, Morton J., Puck, Jennifer M., Bleesing, Jack, Niehues, Tim, Schuetz, Catharina, Malech, Harry, DeRavin, Suk See, Facchetti, Fabio, Gennery, Andrew R., Andersson, Emma, Kamani, Naynesh R., Sekiguchi, JoAnn, Alenezi, Hamid M., Chinen, Javier, Dbaibo, Ghassan, ElGhazali, Gehad, Fontana, Adriano, Pasic, Srdjan, Detre, Cynthia, Terhorst, Cox, Alt, Frederick W., Notarangelo, Luigi D.
Formato: Texto
Lenguaje:English
Publicado: The Rockefeller University Press 2010
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2901061/
https://www.ncbi.nlm.nih.gov/pubmed/20547827
http://dx.doi.org/10.1084/jem.20091927

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