Imino sugar therapy for type 1 Gaucher disease

Glycobiology

Volumn 10, Issue 11, 2000, Pages

  Priestman, D A ^a   Platt, F M ^a   Dwek, R A ^a   Butters, T D ^a  

^a UNIVERSITY OF OXFORD   (United Kingdom)

Author keywords

[No Author keywords available]

Indexed keywords

1 DEOXYNOJIRIMYCIN; ALGLUCERASE; AMINOSUGAR; CARBOHYDRATE DERIVATIVE; GANGLIOSIDE GM2; GLYCOSIDASE; GLYCOSPHINGOLIPID; IMIGLUCERASE; MIGLUSTAT; OLIGOSACCHARIDE; SWAINSONINE;

ANIMAL EXPERIMENT; ANIMAL MODEL; ANTIVIRAL ACTIVITY; CLINICAL TRIAL; CONTROLLED CLINICAL TRIAL; CONTROLLED STUDY; COST EFFECTIVENESS ANALYSIS; DOSE RESPONSE; DRUG COST; DRUG EFFECT; DRUG EFFICACY; DRUG HALF LIFE; DRUG POTENCY; DRUG POTENTIATION; ENZYME ACTIVITY; ENZYME INHIBITION; ENZYME THERAPY; GAUCHER DISEASE; GLYCOGEN METABOLISM; GROWTH REGULATION; HUMAN; LETTER; LIPID METABOLISM; METASTASIS INHIBITION; MOUSE; NONHUMAN; PRIORITY JOURNAL; SANDHOFF DISEASE; TAY SACHS DISEASE;

1-DEOXYNOJIRIMYCIN; ALPHA-GLUCOSIDASES; ANIMALS; ENZYME INHIBITORS; GAUCHER DISEASE; GLUCOSYLCERAMIDASE; GLYCOSPHINGOLIPIDS; HUMANS; MICE;

ANIMALIA;

EID: 0033657474     PISSN: 09596658     EISSN: None     Source Type: Journal    
DOI: None     Document Type: Letter

References (29)

1. Therapeutic response to intravenous infusions of glucocerebrosidase in a patient with Gaucher disease
2. Gaucher disease as a paradigm of current issues regarding single gene mutations of humans
3. Treatment of chronic hepadnavirus infection in a woodchuck animal model with an inhibitor of protein folding and trafficking
4. Purification to homogeneity of Charonia lampas α-fucosidase by using sequential ligand-affinity chromatography
5. Novel oral treatment of Gaucher's disease with N-butyldeoxynojirimycin (OGT-918) to decrease substrate biosynthesis
6. Protein glycosylation in development and disease
7. Glycosidase inhibitors: Inhibitors of N-linked oligosaccharide processing
8. Accelerated transport and maturation of lysosomal α-galactosidase A in Fabry lymphoblasts by an enzyme inhibitor
9. N-butyldeoxynojirimycin-mediated inhibition of human immunodeficiency virus entry correlates with changes in antibody recognition of the V1/V2 region of gp120
10. 3
11. A comparison of the pharmacological properties of carbohydrate remodeled recombinant and placental-derived β-glucocerebrosidase: Implications for clinical efficacy in treatment of Gaucher disease
12. Phase IB clinical trial of the oligosaccharide processing inhibitor swainsonine in patients with advanced malignancies
13. Complex N-glycans in Mgat1 null preimplantation embryos arise from maternal Mgat1 RNA
14. Mice lacking N-acetylglucosaminyltransferase I activity die at mid-gestation, revealing an essential role for complex or hybrid N-linked carbohydrates
15. Glycosylation inhibitors in biology and medicine
16. Delayed symptom onset and increased life expectancy in Sandhoff disease mice treated with N-butyl-deoxynojirimycin
17. The use of inhibitors in the study of glycosidases
18. A genetic model of substrate deprivation therapy for a glycosphingolipid storage disorder
19. A recessive deletion in the Glc-NAc-1-phosphotransferase gene results in peri-implantation embryonic lethality
20. Hepatitis B virus (HBV) envelope glycoproteins vary drastically in their sensitivity to glycan processing: Evidence that alteration of a single N-linked glycosylation site can regulate HBV secretion
21. Complex asparagine-linked oligosaccharides are required for morphogenic events during post-implantation development
22. Inhibitors of glycosphingolipid biosynthesis
23. New therapeutic prospects for the glycosphingolipid lysosomal storage diseases
24. N-Butyldeoxygalactonojirimycin inhibits glycolipid biosynthesis but does not affect N-linked oligosaccharide processing
25. Prevention of lysosomal storage in Tay-Sachs mice treated with N-butyldeoxynojirimycin
26. Extensive glycosphingolipid depletion in the liver and lymphoid organs of mice treated with N-butyldeoxynojirimycin
27. Treatment of Gaucher disease with an enzyme inhibitor
28. Amino-sugar glycosidase inhibitors-versatile tools for glycobiologists
29. Imino sugars inhibit the formation and secretion of bovine viral diarrhea virus, a pestivirus model of hepatitis C virus: Implications for the development of broad spectrum anti-hepatitis virus agents