[zeta] Chain-associated protein of 70 kDa (ZAP70) deficiency in human subjects is associated with abnormalities of thymic stromal cells: Implications for T-cell tolerance

• Published in: Journal of allergy and clinical immunology Vol. 131; no. 2; p. 597
• Main Authors: Poliani, Pietro Luigi, Fontana, Elena, Roifman, Chaim M, Notarangelo, Luigi D
• Format: Journal Article
• Language: English
• Published: St. Louis Elsevier Limited 01.02.2013
• Subjects: Immune system; Mutation; Rodents; T cell receptors
• ISSN: 0091-6749; 1097-6825
• DOI: 10.1016/j.jaci.2012.11.002

AÂ subset of mature mTECs express autoimmune regulator (AIRE), a transcription factor that promotes expression of tissue-restricted antigens, thereby permitting negative selection of T cells recognizing tissue-restricted antigens presented by AIRE+ mTECs and thymic DCs.1 Furthermore, terminally differentiated mTECs in Hassall corpuscles from normal human thymus express involucrin (IVL) and secrete thymic stromal lymphopoietin.2,3 This cytokine acts on myeloid dendritic cells (mDCs) and plasmacytoid dendritic cells (pDCs), which instruct differentiation of natural regulatory T (nTreg) cells expressing the transcription factor forkhead box protein 3 (FOXP3).2 Selection of nTreg cells is also contributed by AIRE+ mTECs. [...]cross-talk between thymocytes and thymic stromal cells is essential to establish immune tolerance.