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PubMed · 16264327

CD3 deficiencies.

Alain Fischer·Geneviève de Saint Basile·Françoise Le Deist
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Abstract

PURPOSE OF REVIEW: The molecular characterization of inherited T-cell immunodeficiencies has contributed to delineating key factors in human T-cell development. This review reports on the recent description of deleterious mutations in the genes encoding CD3 subunits expressed at the T-lymphocyte membrane in association with the T-cell receptor. RECENT FINDINGS: Homozygous mutations in CD3D and CD3E genes lead to a complete block in T-cell development and thus to an early-onset severe combined immunodeficiency phenotype. Thymic studies have shown that the defect in T-cell development occurs at the transition between 'double-negative' and 'double-positive' thymocytes. These results contrast with the partial T-cell immunodeficiency caused by a deficiency in CD3G. SUMMARY: Two new severe combined immunodeficiency conditions have been reported as a consequence of either CD3D or CD3E deficiency. The distinct phenotype of CD3G deficiency sheds light on the differential roles of CD3 subunits in T-lymphocyte development.

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CD3 ComplexHumansLymphopoiesisMutationReceptor-CD3 Complex, Antigen, T-CellSevere Combined ImmunodeficiencyT-LymphocytesThymus GlandJournal ArticleReview

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Alain Fischer, Geneviève de Saint Basile, Françoise Le Deist. 2005. CD3 deficiencies.. https://doi.org/10.1097/01.all.0000191886.12645.79

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