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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
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
2017
Review
2017
Lysosomal storage diseases (LSDs) comprise a large group of disorders of catabolism, mostly due to deficiency of a single glycan… 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
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
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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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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Highly Cited
1997
Highly Cited
1997
The imino sugarN-butyldeoxynojirimycin is an inhibitor of the ceramide-specific glucosyltransferase that catalyzes the first step… Expand
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