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| Author(s): |
Flavia Afonso Lima
Total Authors: 1
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| Document type: | Doctoral Thesis |
| Press: | São Paulo. |
| Institution: | Universidade de São Paulo (USP). Instituto de Ciências Biomédicas (ICB/SDI) |
| Defense date: | 2011-01-26 |
| Examining board members: |
Beatriz Simonsen Stolf Carboni;
Silvia Beatriz Boscardin;
Niels Olsen Saraiva Câmara;
Maria Irma Seixas Duarte;
Wilson Savino
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| Advisor: | Magda Maria Sales Carneiro Sampaio |
| Abstract | |
The thymus is a primary lymphoid organ which is essential for the initial establishment of a functional repertoire of T cells. Notch signaling is crucial for T-cell lineage development from hematopoietic stem cells; however, distribution of Notch ligands and receptors in human thymus is still unknown. AIRE is crucial for the selection of a T-cell-receptor (TCR) repertoire purged of self-reactive specificities. In this study, we analyzed the expression patterns of AIRE and Notch in human thymuses from children with congenital cardiopathies that undergo heart surgery, part of whom with Down syndrome. We described the intra-thymic localization and the cell types that express Notch receptors and ligands. AIRE expression in medullary epithelial cells is significantly decreased in Down syndrome patients. This deficiency could explain higher incidence of autoimmune disease in Down syndrome. (AU) | |