PROMIDISα: A T-cell receptor α signature associated with immunodeficiencies caused by V(D)J recombination defects.

@article{Berland2018PROMIDISAT,
  title={PROMIDISα: A T-cell receptor α signature associated with immunodeficiencies caused by V(D)J recombination defects.},
  author={Aur{\'e}lie Berland and J{\'e}r{\'e}mie Rosain and Sophie Kaltenbach and Vincent Allain and Nizar Mahlaoui and Isabelle Melki and Alice Fi{\'e}vet and Catherine Dubois d'Enghien and Marie Ouach{\'e}e-Chardin and Laurence Perrin and Nathalie Auger and Funda Erol Çipe and Andrea Finocchi and Figen Dogu and Felipe Suarez and Despina Moshous and Thierry Leblanc and Alexandre Belot and Claire Fieschi and David Boutboul and Marion Malphettes and Lionel Galicier and Eric Oksenhendler and St{\'e}phane Blanche and Alain Fischer and Patrick Revy and Dominique Stoppa-Lyonnet and Capucine Picard and Jean-Pierre de Villartay},
  journal={The Journal of allergy and clinical immunology},
  year={2018}
}
BACKGROUND V(D)J recombination ensures the diversity of the adaptive immune system. Although its complete defect causes severe combined immunodeficiency (ie, T-B- severe combined immunodeficiency), its suboptimal activity is associated with a broad spectrum of immune manifestations, such as late-onset combined immunodeficiency and autoimmunity. The earliest… CONTINUE READING