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GLUCOSYLCERAMIDASE

Known as: Lysosomal Glucocerebrosidase, beta Glucocerebrosidase, D-Glucosyl-N-Acylsphingosine Glucohydrolase 
A glycosidase that hydrolyzes a glucosylceramide to yield free ceramide plus glucose. Deficiency of this enzyme leads to abnormally high… 
National Institutes of Health

Related topics

Related topics

28 relations

Narrower (3)

AbcertinGBA1 protein, zebrafishimiglucerase
Acid beta glucosidase:CCnc:Pt:Bld.dot:QnAcid beta glucosidase:CCnt:Pt:Fibroblasts:QnAcid beta glucosidase:CCnt:Pt:WBC:QnAcid beta glucosidase:CCnt:Pt:XXX:Qn

Papers overview

Semantic Scholar uses AI to extract papers important to this topic.
Review
1996
Review
1996
Bioproduction of Human Enzymes in Transgenic Tobacco a
Transgenic plants have significant potential in the bioproduction of complex human therapeutic proteins due to ease of genetic… 
Review
1993
Review
1993
Gaucher disease as a paradigm of current issues regarding single gene mutations of humans.
Gaucher disease is a glycolytic storage disease caused by a deficiency in activity of the catabolic enzyme glucocerebrosidase… 
Highly Cited
1989
Highly Cited
1989
Comparison of RNase A, a chemical cleavage and GC-clamped denaturing gradient gel electrophoresis for the detection of mutations in exon 9 of the human acid beta-glucosidase gene.
Gaucher disease (GD), which results from mutations in the human acid beta-glucosidase (beta-Glc) gene, was used as a model system… 
Highly Cited
1986
Highly Cited
1986
Topology of glucosylceramide synthesis in Golgi membranes from porcine submaxillary glands.
Highly Cited
1984
Highly Cited
1984
Structure of the N-asparagine-linked oligosaccharide units of human placental beta-glucocerebrosidase.
The N-asparagine-linked oligosaccharide chains of homogeneous human placental beta-glucocerebrosidase were released by… 
Highly Cited
1982
Highly Cited
1982
Mutations of glucocerebrosidase: discrimination of neurologic and non-neurologic phenotypes of Gaucher disease.
Multiple molecular forms of beta-glucocerebrosidase that permit discrimination between neurologic and non-neurologic phenotypes… 
Highly Cited
1981
Highly Cited
1981
Purification and characterization of a cytosolic broad specificity beta-glucosidase from human liver.
Highly Cited
1977
Highly Cited
1977
Enzyme replacement therapy in Gaucher's disease: large-scale purification of glucocerebrosidase suitable for human administration.
Enzyme replacement therapy for the alleviation of Gaucher's disease has been impeded because of the difficulty in preparing large… 
Highly Cited
1973
Highly Cited
1973
Isolation and characterization of glucocerebrosidase from human placental tissue.
Abstract Human placental glucocerebrosidase was purified 4000-fold to apparent homogeneity. The most highly enriched preparation… 
Highly Cited
1972
Highly Cited
1972
Adult Gaucher's disease: kindred studies and demonstration of a deficiency of acid beta-glucosidase in cultured fibroblasts.
Two phenotypically different forms of Gaucher's disease exist, a chronic non-cerebral form (adult Gaucher's disease) and an… 
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