Thymic Epithelium Abnormalities in DiGeorge and Down Syndrome Patients Contribute to Dysregulation in T Cell Development

Thymic Epithelium Abnormalities in DiGeorge and Down Syndrome Patients Contribute to Dysregulation in T Cell Development

The thymus plays a fundamental role in establishing and maintaining central and peripheral tolerance and defects in thymic architecture or AIRE expression result in the development of autoreactive lymphocytes. Patients with partial DiGeorge Syndrome (pDGS) and Down Syndrome (DS) present alterations...

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Main Authors: Genni Enza Marcovecchio, Ileana Bortolomai, Francesca Ferrua, Elena Fontana, Luisa Imberti, Erika Conforti, Donato Amodio, Sonia Bergante, Giulia Macchiarulo, Veronica D'Oria, Francesca Conti, Silvia Di Cesare, Georgia Fousteri, Adriano Carotti, Alessandro Giamberti, Pietro Luigi Poliani, Luigi D. Notarangelo, Caterina Cancrini, Anna Villa, Marita Bosticardo
Format: Article
Language:English
Published: Frontiers Media S.A. 2019-03-01
Series:Frontiers in Immunology
Subjects:
thymus
DiGeorge syndrome
Down syndrome
regulatory T (Treg) cells
thymocytes
Online Access:https://www.frontiersin.org/article/10.3389/fimmu.2019.00447/full

Internet

https://www.frontiersin.org/article/10.3389/fimmu.2019.00447/full

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