Human CD3γ, but not CD3δ, haploinsufficiency differentially impairs γδ versus αβ surface TCR expression.

Munoz-Ruiz M., Perez-Flores V., Garcillan B., Guardo A. C., Mazariegos M. S., Takada H., ...More

BMC immunology, vol.14, pp.3, 2013 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 14
  • Publication Date: 2013
  • Doi Number: 10.1186/1471-2172-14-3
  • Journal Name: BMC immunology
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.3
  • Keywords: T cells, CD3, Haploinsufficiency, SEVERE COMBINED IMMUNODEFICIENCY, CELL RECEPTOR-CD3 COMPLEX, TCR/CD3 COMPLEX, T-CELLS, LYMPHOCYTES, CHAINS
  • Bursa Uludag University Affiliated: Yes

Abstract

Background: The T cell antigen receptors (TCR) of a beta and gamma delta T lymphocytes are believed to assemble in a similar fashion in humans. Firstly, alpha beta or gamma delta TCR chains incorporate a CD3 delta epsilon dimer, then a CD3 delta epsilon dimer and finally a zeta zeta homodimer, resulting in TCR complexes with the same CD3 dimer stoichiometry. Partial reduction in the expression of the highly homologous CD3 gamma and CD3 delta proteins would thus be expected to have a similar impact in the assembly and surface expression of both TCR isotypes. To test this hypothesis, we compared the surface TCR expression of primary alpha beta and gamma delta T cells from healthy donors carrying a single null or leaky mutation in CD3G (gamma(+/-)) or CD3D (delta(+/-), delta(+/leaky)) with that of normal controls.