Flow cytometric assays to detect platelet activation and aggregation in device-implanted calves |
| |
Authors: | LC Baker WC Davis J Autieri MJ Watach K Yamazaki P Litwak WR Wagner |
| |
Affiliation: | Unité INSERM 429, H?pital Necker-Enfants Malades, 75743 Paris, Cedex 15, France. rieux@necker.fr |
| |
Abstract: | Omenn's syndrome is an inherited human combined immunodeficiency condition characterized by the presence of a large population of activated and tissue-infiltrating T cells. Analysis of the TCRB repertoire revealed a highly restricted TCRBV usage in three patients. More strikingly, T cell clones from the three patients expressed TCRB chains with VDJ junction similarities, suggesting a common antigenic specificity. Analysis of the TCRA repertoire in one patient also revealed a restricted TCRAV usage. Finally, analysis of the TCRBV repertoire of tissue-infiltrating T cells in one patient suggested nonrandom tissue migration. These results suggest that the oligoclonal expansion of T cells observed in Omenn's syndrome could be the consequence of autoimmune proliferation generated by a profound defect in lymphocyte development. |
| |
Keywords: | |
本文献已被 PubMed 等数据库收录! |
|