%0 Journal Article
%A Marín Marín, Ana Victoria
%A Garcillán Goyoaga, Beatriz de
%A Jiménez Reinoso, Anaïs
%A Muñoz Ruiz, Miguel
%A Briones Contreras, Alejandro
%A Fernández Malavé, Edgar
%A Recio Hoyas, María José
%A Regueiro González-Barros, José Ramón
%T Human congenital T-cell receptor disorders
%D 2015
%@ 2292-5937; 2292-5945
%U https://hdl.handle.net/20.500.14352/34395
%X Immunodeficiencies of most T-cell receptor (TCR) components (TCRID) have been reported in almost 40 patients worldwide who have also, at times, shown signs of autoimmunity. We updated their clinical, immunological, and molecular features with an emphasis on practical diagnosis, as the range of the disorder grows in complexity with new partial defects. Cellular and animal models are also reviewed and in some cases reveal their limitations for predicting TCRID immunopathology.
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