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

doi:10.1084/jem.20091927

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Article

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

The Journal of experimental medicine - 5 Jul 2010

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

Abstract excerpt

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...

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Identifiers

PMID: 20547827
DOI: 10.1084/jem.20091927
PMCID: PMC2901061

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 2 Sept 2026. Prepared record publication-20547827-2026-09-02.2. Manifest publication-graph-cohort-2026-09-02.2.

Source receipt: PubMed via Helena Literature Corpus, retrieved 2 Sept 2026.

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