Molecular analysis of T-cell receptor (TCR) repertoire, by measuring the CDR3 heterogeneity length of beta-variable regions (spectratyping), is useful for acquiring novel information on the status of immune system in primary immunodeficiency. Here, we evaluate TCR repertoire in a child with trichothiodystrophy (TTD) and combined immunodeficiency (CID). Spectratyping revealed marked alterations of TCR repertoire distribution: 21 and 10 out of 27 TCR Vbeta (TCRBV) families and subfamilies were skewed in CD8(+) and CD4(+) subsets, respectively. These findings revealed, for the first time in a TTD patient with CID, a marked reduction in the TCR repertoire complexity, which may reflect alterations in the mechanisms regulating the generation and homeostasis of T cells.
Cancrini C., R.M. (2002). Restriction in T-cell receptor repertoire in a patient affected by trichothiodystrophy and CD4+ lymphopenia. Scandinavian Journal of Immunology, 56(2), 212-216.
Tipologia: | Articolo su rivista | |
Citazione: | Cancrini C., R.M. (2002). Restriction in T-cell receptor repertoire in a patient affected by trichothiodystrophy and CD4+ lymphopenia. Scandinavian Journal of Immunology, 56(2), 212-216. | |
Lingua: | English | |
Settore Scientifico Disciplinare: | Settore MED/38 - Pediatria Generale e Specialistica | |
Revisione (peer review): | Sì, ma tipo non specificato | |
Tipo: | Articolo | |
Rilevanza: | Rilevanza internazionale | |
Digital Object Identifier (DOI): | http://dx.doi.org/10.1046/j.1365-3083.2002.01122.x | |
Stato di pubblicazione: | Pubblicato | |
Data di pubblicazione: | 2002 | |
Titolo: | Restriction in T-cell receptor repertoire in a patient affected by trichothiodystrophy and CD4+ lymphopenia | |
Autori: | ||
Autori: | Cancrini C., Romiti M.L., Di Cesare S., Angelini F., Gigliotti D., Livadiotti S., Bertini E., Rossi P., Racioppi L. | |
Appare nelle tipologie: | 01 - Articolo su rivista |