Anomalies d’expression du complexe récepteur T de l’antigène/CD3 et déficits immunitaires
Bibliographic record
Abstract
Molecular characterization of immunodeficiencies contributes to a better understanding of the physiological mechanisms of immune function. The T cell receptor is a heterodimer (alpha/beta or gamma/delta) associated with four transmembrane units of the CD3 complex (gamma, delta, epsilon and zeta). We herein summarize the immunodeficiency states resulting from defects in genes encoding the CD3 complex. Such analysis highlights the respective role of each of these chains in T lymphocyte development and underscores differences between T lymphocyte development in man and mouse. Currently, there is a growing body of knowledge on immunodeficiencies specifically involving the four chains of the CD3, namely gamma, delta, epsilon and zeta. Thus, we can compare the phenotypes observed in these patients with those seen in mice knockout for these genes. The main differences observed involve the respective roles of the CD3gamma chain as well as the CD3delta, whose functions seem to be reciprocal between the two species. Indeed, in the mouse, knockout of CD3delta allows some degree of T lymphocyte differentiation since mature CD4 and CD8 as well as TCRgammadelta T lymphocytes are observed in the periphery. In contrast, deleterious mutation of the CD3delta encoding gene in the human leads to a severe combined immunodeficiency characterised by the complete absence of mature T cell subpopulations including TCRalpha/beta and TCRgamma/delta. Reciprocally, in the human, mutation of the CD3gamma encoding gene leads to a moderate immunodeficiency which contrasts with the complete block of T cell differentiation observed in mice knockout for this gene. This article brings into focus the knowledge gained through studies of immunodeficiency mouse models with the pathophysiological state observed in human disease.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.001 |
| Meta-epidemiology (narrow) | 0.001 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.001 | 0.000 |
| Science and technology studies | 0.000 | 0.001 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.001 | 0.001 |
| Insufficient payload (model declined to judge) | 0.002 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".