Skewed T-cell receptor repertoire, decreased thymic output, and predominance of terminally differentiated T cells in ataxia telangiectasia.

@article{Giovannetti2002SkewedTR,
  title={Skewed T-cell receptor repertoire, decreased thymic output, and predominance of terminally differentiated T cells in ataxia telangiectasia.},
  author={Antonello Giovannetti and Francesca Mazzetta and E. J. Caprini and Alessandro Aiuti and Marco Marziali and Marina Pierdominici and Andrea Cossarizza and Luciana Chessa and Enrico Scala and Isabella Quinti and Giandomenico Russo and Massimo Fiorilli},
  journal={Blood},
  year={2002},
  volume={100 12},
  pages={4082-9}
}
Ataxia telangiectasia (A-T), a genetic disorder caused by the homozygous mutation of the ATM gene, frequently associates with variable degrees of cellular and humoral immunodeficiency. However, the immune defects occurring in patients with A-T are still poorly characterized. Here we show that the T-cell receptor (TCR) variable beta (BV)-chain repertoire of 9 A-T patients was restricted by diffuse expansions of some variable genes prevalently occurring within the CD4 subset and clustering to… CONTINUE READING

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