Primary T-cell immunodeficiencies

Curr Opin Immunol. 1993 Aug;5(4):569-78. doi: 10.1016/0952-7915(93)90040-y.

Abstract

The phenotypes of many primary T-cell immunodeficiencies have been described, including diseases associated with defective T-cell differentiation and/or activation. Recently, genotypes have been defined for some of them, such as X-linked severe combined immunodeficiency and CD3 deficiencies (or hyper IgM syndrome). Phenotype/genotype correlation studies open a fruitful way to a better understanding of primary T-cell immunodeficiencies.

Publication types

  • Review

MeSH terms

  • Adenosine Deaminase / deficiency
  • CD3 Complex / genetics
  • DNA Repair / genetics
  • DiGeorge Syndrome / genetics
  • Genetic Linkage
  • Histocompatibility Antigens Class II / genetics
  • Humans
  • Immunologic Deficiency Syndromes / genetics*
  • Lymphocyte Activation / genetics
  • Severe Combined Immunodeficiency / genetics
  • T-Lymphocytes
  • Wiskott-Aldrich Syndrome / genetics
  • X Chromosome

Substances

  • CD3 Complex
  • Histocompatibility Antigens Class II
  • Adenosine Deaminase