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Accueil > Publications > Recherche par années > Années 1990 > 1991

Piller, F ; Ledeist, F ; Weinberg, KI ; Parkman, R ; Fukuda, M

Altered o-glycan synthesis in lymphocytes from patients with wiskott-aldrich syndrome

Journal of Experimental Medicine 173 (6) 1501-1510

par Administrateur - publié le

Abstract :

The only molecular defect reported for the X-linked immunodeficiency Wiskott-Aldrich syndrome (WAS) is the abnormal electrophoretic behavior of the major T lymphocyte sialoglycoprotein CD43. Since the 70 to 80 O-linked carbohydrate chains of CD43 are known to influence markedly its electrophoretic mobility, we analyzed the structure and the biosynthesis of O-glycans of CD43 in lymphocytes from patients with WAS. Immunofluorescence analysis with the carbohydrate dependent anti-CD43 antibody T305 revealed that in 10 out of the 12 WAS patients tested increased numbers of T lymphocytes carry on CD43 an epitope which on normal lymphocytes is expressed only after activation.