Browse by author
Lookup NU author(s): Professor Hermann Josef Vormoor
Full text for this publication is not currently held within this repository. Alternative links are provided below where available.
Leukocyte adhesion deficiency II has been described in only 2 patients; herein we report extensive investigation of another patient. The physical stigmata were detected during prenatal ultrasonographic investigation. Sialyl-Lewis X (sLex) was absent from the surface of polymorphonuclear neutrophils, and cell binding to E- and P-selectin was severely impaired, causing an immunodeficiency. The elevation of peripheral neutrophil counts occurred within several days after birth. A severe hypofucosylation of glycoconjugates bearing fucose in different glycosidic links was present in all cell types investigated, demonstrating that leukocyte adhesion deficiency II is not only a disorder of leukocytes but a generalized inherited metabolic disease affecting the metabolism of fucose.
Author(s): Marquardt T, Brune T, Lühn K, Zimmer KP, Körner C, Fabritz L, van der Werft N, Vormoor J, Freeze HH, Louwen F, Biermann B, Harms E, von Figura K, Vestweber D, Koch HG
Publication type: Article
Publication status: Published
Journal: Journal of Pediatrics
Print publication date: 01/06/1999
ISSN (print): 0022-3476
ISSN (electronic): 1097-6833
Notes: 0022-3476 (Print)
Altmetrics provided by Altmetric