Miglustat

Known as: Miglustatum, N-Butyl deoxynojirimycin, 1,5-(butylimino)-1,5-dideoxy-D-glucitol 
A synthetic, N-alkylated imino analogue of D-glucose. Miglustat competitively and reversibly binds to and inhibits the activity of UDP-glucose… (More)
National Institutes of Health

Topic mentions per year

Topic mentions per year

1954-2017
010203019542016

Papers overview

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Highly Cited
2010
Highly Cited
2010
A randomized, controlled trial of miglustat indicated that miglustat (Zavesca) stabilized neurological disease over 12 months in… (More)
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Highly Cited
2010
Highly Cited
2010
Niemann-Pick disease type C is a rare, genetic disease associated with impaired intracellular lipid trafficking and progressive… (More)
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Highly Cited
2008
Highly Cited
2008
OBJECTIVE To evaluate the efficacy and safety of miglustat, concomitant with enzyme replacement therapy (ERT), in patients with… (More)
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Highly Cited
2007
Highly Cited
2007
BACKGROUND Niemann-Pick type C disease (NPC) is an inherited neurodegenerative disorder characterised by an intracellular lipid… (More)
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Highly Cited
2004
Highly Cited
2004
Summary: It has been shown that treatment with miglustat (Zavesca, N-butyldeoxynojirimycin, OGT 918) improves key clinical… (More)
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Highly Cited
2004
Highly Cited
2004
Niemann-Pick disease type C (NP-C) is a hereditary neurovisceral lipid storage disorder. Although traditionally considered a… (More)
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Highly Cited
2001
Highly Cited
2001
Niemann-Pick type C (NPC) disease is a cholesterol lipidosis caused by mutations in NPC1 and NPC2 gene loci. Most human cases are… (More)
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Highly Cited
2000
Highly Cited
2000
BACKGROUND Current treatment for Gaucher's disease involves administration of intravenous glucocerebrosidase to degrade… (More)
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Highly Cited
1997
Highly Cited
1997
The glycosphingolipid (GSL) lysosomal storage diseases result from the inheritance of defects in the genes encoding the enzymes… (More)
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Highly Cited
1994
Highly Cited
1994
The imino sugar deoxynojirimycin and its alkylated derivatives are inhibitors of the N-linked oligosaccharide processing enzymes… (More)
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