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Factors Affecting the Binding of Glucosylceramidase to ItsNatural Substrate Dispersion
This paper reports the results of ultracentrifugation experiments devised for investigating the interactions occurring in the conditions of the enzymatic assay between glucosylceramidase and the components of the substrate dispersion. This dispersion contains, besides glucosylceramide, taurocholate and oleic acid.
Anna Maria Vaccaro +3 more
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Comparison of synthetic and natural glucosylceramides as substrate for glucosylceramidase assay
Clinica Chimica Acta, 1982Commercially available [3H]glucosylceramide is derived from spleen tissue of patients with Gaucher's disease. When such tritiated glucosylceramide was diluted with unlabelled glucosylceramide from different sources and used as the substrate for assays of glucosylceramidase, the apparent activities obtained differed drastically.
A M, Vaccaro, T, Kobayashi, K, Suzuki
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Glucosylceramidase (EC 3.2.1.45) is a membrane-bound, lysosomal enzyme, which hydrolyzes glucosylceramide into glucose and ceramide (1). As for several other glycosphingolipid hydrolses, also for glucosylceramidase a special activator protein capable of stimulating its activity has been described in the past (2–5).
Anna Maria Vaccaro +4 more
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International Journal of Peptide Research and Therapeutics, 2008
The mutations in human glucosylceramidase lead to Gaucher disease, which is the most prevalent lysosomal storage disease. So far 153 point mutations have been recorded in human glucosylceramidase, resulting in a wide variability in clinical presentations.
Shaomin Yan, Guang Wu
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The mutations in human glucosylceramidase lead to Gaucher disease, which is the most prevalent lysosomal storage disease. So far 153 point mutations have been recorded in human glucosylceramidase, resulting in a wide variability in clinical presentations.
Shaomin Yan, Guang Wu
exaly +2 more sources
A New Glucosylceramidase Activator in Human Placenta
1986Natural substrates of many lysosomal hydrolases are highly hydrophobic. While the reactions can proceed in vitro when appropriate detergents are included in the assay mixture, these enzymes must function in vivo without artificial detergents of high concentrations often required for in vitro reactions.
Anna Maria Vaccaro +5 more
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Etiology of a New Identified Gaucher Disease Variant without Glucosylceramidase Defect
Gaucher disease, the most prevalent lysosomal storage disease, is a group of autosomal recessively inherited disorders characterized by the accumulation of glucosylceramide in lysosomes of the cells of the reticuloendothelial system. This sphingolipidosis is caused by the hereditary deficiency of the membran associated lysosomal enzyme β ...
Helen Christomanou +3 more
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Parkinson's disease (PD) is a neurodegenerative disorder affecting 2-3% of those aged over 65, characterized by motor symptoms like slow movement, tremors, and muscle rigidity, along with non-motor symptoms such as anxiety and dementia. Lewy bodies, clumps of misfolded proteins, contribute to neuron loss in PD. Mutations in the GBA1 gene are considered
Júlio César Claudino dos Santos +12 more
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