Patients with LRBA deficiency show CTLA4 loss and immune dysregulation responsive to abatacept therapy

Lo, Bernice; Zhang, Kejian; Lü, Wei; Zheng, Lixin; Zhang, Qian; Kanellopoulou, Chrysi; Zhang, Yu; Liu, Zhiduo; M. Fritz, Jill; Marsh, Rebecca; Husami, Ammar; Kissell, Diane; Nortman, Shannon; Chaturvedi, Vijaya; Haines, Hilary; R. Young, Lisa; Mo, Jun; H. Filipovich, Alexandra; Bleesing, Jack; Mustillo, Peter; C. Stephens, Michael; M. Rueda, Cesar; Chougnet, Claire; Hoebe, Kasper; McElwee, Joshua; D. Hughes, Jason; Karakoç-Aydıner, Elif; Matthews, Helen; Price, Susan; C. Su, Helen; Koneti Rao, V.; J. Lenardo, Michael; B. Jordan, Michael

doi:10.1126/science.aaa1663

Public

Patients with LRBA deficiency show CTLA4 loss and immune dysregulation responsive to abatacept therapy

Published in Science • Jul 23, 2015

Authors:

Bernice Lo

Kejian Zhang

Wei Lü

Abstract

Mutations in the LRBA gene (encoding the lipopolysaccharide-responsive and beige-like anchor protein) cause a syndrome of autoimmunity, lymphoproliferation, and humoral immune deficiency. The biological role of LRBA in immunologic disease is unknown. We found that patients with LRBA deficiency manif...

Subject

Abatacept

Immune dysregulation

Medicine

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