[HTML][HTML] A new type of carbohydrate-deficient glycoprotein syndrome due to a decreased import of GDP-fucose into the Golgi
T Lübke, K von Figura, C Körner, T Marquardt - Journal of Biological …, 1999 - ASBMB
The fucosylation of glycoproteins was found to be deficient in a patient with a clinical
phenotype resembling that of leukocyte adhesion deficiency type II (LAD II). While in LAD II
hypofucosylation of glycoconjugates is secondary to an impaired synthesis of GDP-fucose
due to a deficiency of the GDP-d-mannose-4, 6-dehydratase, synthesis of GDP-fucose was
normal in our patient (Körner, C., Linnebank, M., Koch, H., Harms, E., von Figura, K., and
Marquardt, T.(1999) J. Leukoc. Biol., in press). Import of GDP-fucose into Golgi-enriched …
phenotype resembling that of leukocyte adhesion deficiency type II (LAD II). While in LAD II
hypofucosylation of glycoconjugates is secondary to an impaired synthesis of GDP-fucose
due to a deficiency of the GDP-d-mannose-4, 6-dehydratase, synthesis of GDP-fucose was
normal in our patient (Körner, C., Linnebank, M., Koch, H., Harms, E., von Figura, K., and
Marquardt, T.(1999) J. Leukoc. Biol., in press). Import of GDP-fucose into Golgi-enriched …
