No abstract

The agencies that provided financial support for the conduct of the research and preparation of the article played no role in the study design; in the collection, analysis and interpretation of data; in the writing of the report, or in the decision to submit the article for publication.

Bl azquez Moreno's institution has received grant funding from MINEICO (SVP-2014-068263). A. del Pozo Mat e has received consultancy fees from ENAC and lecture fees from the Health Institute Carlos III. E. Vallesp ın Garc ıa has received funds for expert testimony for the Scienceboard.net and royalties from KARYOARRAY-8.512.907. M. Val es-G omez's institution has received grant funding from MINEICO (SAF2015-69169-R) and Comunidad de Madrid (S2010/BMD-2326). H. T. Reyburn's institution has received a grant from MINEICO (SAF2014-58752-R). The rest of the authors declare that they have no relevant conflicts of interest.

No abstract

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.