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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

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Highly Cited
1995
Highly Cited
1995
Growth factors and stromal support generate very efficient retroviral transduction of peripheral blood CD34+ cells from Gaucher patients.
We have achieved high-efficiency gene transfer into nonmobilized peripheral blood (PB) CD34+ cells from patients with Gaucher's… 
Highly Cited
1994
Highly Cited
1994
Identification of Glu340 as the active-site nucleophile in human glucocerebrosidase by use of electrospray tandem mass spectrometry.
Gaucher disease is an inherited lysosomal storage disorder caused by a deficiency of human acid beta-glucosidase… 
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
1985
Highly Cited
1985
The subcellular localization of soluble and membrane-bound lysosomal enzymes in I-cell fibroblasts: a comparative immunocytochemical study.
Using electron microscopic immunocytochemistry with gold probes, we have studied the localization of acid alpha-glucosidase, N… 
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
1984
Highly Cited
1984
Studies on the mechanism of castanospermine inhibition of α- and β-glucosidases
1983
1983
Modification of subunit interaction in membrane‐bound acid β‐glucosidase from Gaucher disease
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
1977
Highly Cited
1977
Mass Spectroscopic Identification of Cytokinins: Glucosyl Zeatin and Glucosyl Ribosylzeatin from Vinca rosea Crown Gall.
Mass spectrographic and chemical studies of the permethyl and trimethylsilyl ethers of two new cytokinins isolated from Vinca… 
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