glucosylceramidase activity

Known as: ceramide glucosidase activity, glucosylsphingosine beta-glucosidase activity, acid beta-glucosidase activity 
Catalysis of the reaction: D-glucosyl-N-acylsphingosine + H2O = D-glucose + N-acylsphingosine. [EC:3.2.1.45]
National Institutes of Health

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2015
2015
Glucocerebrosidase (GBA) mutations have been associated with Parkinson's disease in numerous studies. However, it is unknown… (More)
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2015
2015
The principal risk factor for developing most adult onset neurodegenerative diseases is aging, with incidence rising… (More)
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Highly Cited
2014
Highly Cited
2014
Gaucher disease is caused by mutations in the glucocerebrosidase gene, which encodes the lysosomal hydrolase glucosylceramidase… (More)
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Highly Cited
2011
Highly Cited
2011
Emerging genetic and clinical evidence suggests a link between Gaucher disease and the synucleinopathies Parkinson disease and… (More)
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2009
2009
The autophagy-lysosomal degradation pathway plays a role in the onset and progression of neurodegenerative diseases. Clinical and… (More)
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2008
2008
The main clinical features of two siblings from a consanguineous marriage were progressive myoclonic epilepsy without… (More)
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2007
2007
The primary catabolic pathway for glucosylceramide is catalyzed by the lysosomal enzyme glucocerebrosidase that is defective in… (More)
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1995
1995
Three sisters suffering from an unusual form of Gaucher's disease are described. These patients had cardiovascular abnormalities… (More)
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1994
1994
To elucidate the mechanisms that are involved in the decrease of ceramide levels in atopic dry skin and in aged skin, we examined… (More)
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1992
Highly Cited
1992
GAUCHER'S disease is the most prevalent lysosomal storage disorder in humans and results from an autosomally inherited deficiency… (More)
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