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American Society of Hematology, Blood, 25(125), p. 3886-3895, 2015

DOI: 10.1182/blood-2014-12-618363

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Fatal autoimmunity in mice reconstituted with human hematopoietic stem cells encoding defective FOXP3

Journal article published in 2015 by Jeremy A. Goettel, Subhabrata Biswas, Willem S. Lexmond, Ada Yeste, Laura Passerini, Bonny Patel, Siyoung Yang, Jiusong Sun, Jodie Ouahed, Dror S. Shouval, Katelyn J. McCann, Bruce H. Horwitz, Diane Mathis, Edgar L. Milford, Luigi D. Notarangelo and other authors.
This paper is made freely available by the publisher.
This paper is made freely available by the publisher.

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Abstract

Key PointsImproved adaptive immune responses in humanized mice lacking murine MHC II and expressing human HLADR1. NOD.PrkdcscidIl2rγ−/−H2-Ab1−/− Tg(HLA-DR1) mice reconstituted with hematopoietic stem cells from an IPEX syndrome patient develop fatal autoimmunity.