N-butyldeoxygalactonojirimycin: a more selective inhibitor of glycosphingolipid biosynthesis than N-butyldeoxynojirimycin, in vitro and in vivo

LYSOSOMAL STORAGE DISEASES

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Marked elevation of plasma chitotriosidase activity. A novel hallmark of Gaucher disease.

Carla E. M. Hollak, Sonja van Weely, M. H. van Oers et al. · Journal of Clinical Investigation · 1994 · 899 citations · Full text

Clinical Symptoms, Immunology, Glycobiology +17

N-butyldeoxynojirimycin is a novel inhibitor of glycolipid biosynthesis.

Frances M. Platt, Gabrielle R. Neises, Raymond A. Dwek et al. · Journal of Biological Chemistry · 1994 · 451 citations · Full text

Medicinal Chemistry, Biosynthesis, Bioorganic Chemistry +14

Enzyme Therapy in Type 1 Gaucher Disease: Comparative Efficacy of Mannose-Terminated Glucocerebrosidase from Natural and Recombinant Sources

Gregory A. Grabowski · Annals of Internal Medicine · 1995 · 444 citations

Therapeutic response to intravenous infusions of glucocerebrosidase in a patient with Gaucher disease.

Norman W. Barton, F S Furbish, Gary J. Murray et al. · Proceedings of the National Academy of Sciences · 1990 · 370 citations · Full text

Nutrition, Glycobiology, Immunology +19