Immunodeficiency Associated with Defective Expression of the T-Cell Receptor–CD3 Complex

Publisher Website
Google Scholar
Abstract
In the November 3, 1988, issue of the Journal, Alarcon et al. reported on a familial defect in the surface expression of the T-cell receptor—CD3 complex leading to an impaired immune response in two siblings.1 The biochemical studies showed that the intracellular expression of the CD3–ζ chain of the T-cell receptor was reduced, whereas all other components were normally expressed. The authors concluded that the decreased association of the ζ chain with the other proteins of the complex was the primary defect responsible for the low surface expression of the T-cell receptor—CD3 complex.