Decreased alpha/beta heterodimer among CD8 molecules of peripheral blood T cells in Wiskott-Aldrich syndrome.
Cell surface CD8 molecules exist as either alpha/alpha homodimers or alpha/beta heterodimers, and most CD8+ T cells that undergo differentiation in the thymus express alpha/beta heterodimers. CD8 molecules on peripheral blood lymphocytes of patients with Wiskott-Aldrich syndrome (WAS), an X-linked immunodeficiency disease, have now been shown to be composed mostly of alpha/alpha homodimers, while the total number of lymphocytes was maintained. This observation may indicate aberrant differentiation of CD8+ T cells in the thymus of WAS. Decreased CD8alpha/beta heterodimer expression, and the relative increase of CD8+ T cells expressing CD8alpha/alpha homodimer, may explain a part of the defective lymphocyte functions in WAS patients.
Kawabata
K
K
Department of Pediatrics, School of Medicine, Tokyo Medical and Dental University, Japan.
Nagasawa
M
M
Morio
T
T
Okawa
H
H
Yata
J
J
D000375:Aging / Q000502:physiology; D017252:CD3 Complex / Q000032:analysis; D018414:CD8-Positive T-Lymphocytes / Q000737:chemistry / Q000166:cytology*; D005434:Flow Cytometry / Q000379:methods; D006801:Humans; D007136:Immunoglobulins / Q000097:blood; D007223:Infant; D007694:Killer Cells, Natural / Q000166:cytology; D018655:Lymphocyte Count; D016693:Receptors, Antigen, T-Cell, alpha-beta / Q000032:analysis; D014923:Wiskott-Aldrich Syndrome / Q000097:blood / Q000276:immunology / Q000473:pathology*
D017252:CD3 Complex; D007136:Immunoglobulins; D016693:Receptors, Antigen, T-Cell, alpha-beta