Marín Marín, Ana VictoriaGarcillán Goyoaga, Beatriz deJiménez Reinoso, AnaïsMuñoz Ruiz, MiguelBriones Contreras, AlejandroFernández Malavé, EdgarRecio Hoyas, María JoséRegueiro González-Barros, José Ramón2023-06-192023-06-1920152292-5937; 2292-5945dx.doi.org/10.14785/lpsn-2014-0012https://hdl.handle.net/20.500.14352/34395Immunodeficiencies 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.engjournal articlehttps://doi.org/10.14785/lpsn-2014-0012restricted accessT-cell receptorImmunodeficienciesInmunología2412 Inmunología