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glycosphingolipidoses

 
National Institutes of Health

Papers overview

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2020
2020
Glycosphingolipids (GSLs) exist exclusively in the outer leaflet of plasma membrane in mammalian cells and have diverse… Expand
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Review
2019
Review
2019
Glycosphingolipids (GSLs), the main topic of this review, are a subclass of sphingolipids. With their glycans exposed to the… Expand
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Review
2012
Review
2012
A viable treatment for lysosomal storage disease has been very difficult to attain. One option is pharmacological inhibition of… Expand
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2009
2009
Objectives and background The iminosugar N-(5-adamantane-1-ylmethoxy)pentyl-1-deoxynojirimycin (AMPDNM) potently controls… Expand
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Review
2005
Review
2005
The inherited metabolic disorders of glycosphingolipid (GSL) metabolism are a relatively rare group of diseases that have diverse… Expand
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Review
2004
Review
2004
The glycosphingolipid lysosomal storage diseases are a group of monogenic human disorders caused by the impaired catalytic… Expand
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Review
2004
Review
2004
Glycosphingolipids are ubiquitous membrane constituents that are subdivided in neutral or acidic fractions (gangliosides and… Expand
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Highly Cited
2000
Highly Cited
2000
Deficiency of lysosomal acid beta-glucosidase induces glycolipid storage in the macrophages of Gaucher disease but the pathways… Expand
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Review
1999
Review
1999
Abstract The glycosphingolipidoses are a set of diseases that are caused by defects in the lysosomal degradation of glycolipids… Expand
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1997
1997
The imino sugar N-butyldeoxynojirimycin is an inhibitor of the ceramide-specific glucosyltransferase that catalyzes the first… Expand
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