16 search results for “glucocerebrosidase” in the Public website
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Glucocerebrosidase and glycolipids: In and beyond the lysosome
The lysosomal β-glucosidase named glucocerebrosidase (GCase) is a retaining β-glucosidase that hydrolyzes the glycosphingolipid glucosylceramide (GlcCer) to ceramide and glucose at acid pH.
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Ultrasensitive in situ visualization of active glucocerebrosidase molecules
Deficiency of glucocerebrosidase (GBA) underlies Gaucher disease, a common lysosomal storage disorder. Carriership for Gaucher disease has recently been identified as major risk for parkinsonism. Presently, no method exists to visualize active GBA molecules in situ. We here report the design, synthesis…
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Chemical biology of glucosylceramide metabolism fundamental studies and applications for Gaucher disease
This thesis describes biochemical investigations of glucocerebrosidase (GBA), the lysosomal β- glucosidase that is deficient in Gaucher disease (GD).
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Understanding functional dynamics and conformational stability of beta-glycosidases
Due to their central physiological roles in living organisms, retaining beta-glycosidases have been the subject of tremendous research efforts to examine their structure/function relation using numerous biophysical and biochemical approaches.
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Lindsey LelieveldScience
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Applications for activity-based probes in biomedical research on glycosidases
Lysosomal glycosidases are acid hydrolases that fragment glycoconjugates in lysosomes.
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Glucocerebrosidase and Glycolipids
PhD defence
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Martijn van der LiendenScience
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Zebrafish as vertebrate model of Gaucher disease
Lysosomal storage disorders (LSDs) are a group of orphan diseases characterized by lysosomal dysfunction or impaired lysosomal catabolism and affect collectively about 1 in 5000 live births.
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Investigations on the role of impaired lysosomes of macrophages in disease
The research described in this thesis combines the latest insights in lysosomal function with lysosome centred cell signalling.
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Activity-based protein profiling of glucosidases, fucosidases and glucuronidases
Promotores: H.S. Overkleeft, G.A. van der Marel, Co-promotor: B.I. Florea
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Research
Through metabolism, biochemical processes give rise to the complexity of life. Acquired and inborn errors in metabolism underlie many diseases occurring in man. The challenge for present day medical biochemistry is to find, and integrate, pieces of information at molecular, cell and organismal level…
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Fundamental and translational medical biochemistry
Through metabolism, biochemical processes give rise to the complexity of life. Acquired and inborn errors in metabolism underlie many diseases occurring in man. The challenge for present day medical biochemistry is to find, and integrate, pieces of information at molecular, cell and organismal level…
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Projects
Research projects
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Changing enzymes for clean energy and disease prevention
They can play a role in metabolic disorders and can break down tough plant fibers: β-glycosidases are enzymes that play many roles in nature. Fredj Ben Bdira changed these enzymes in order to enhance the production of clean energy and to improve the treatment of patients with metabolic diseases.
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Activity-based protein profiling for drug discovery
Activity-based protein profiling (ABPP, also termed chemical proteomics), is one of the pillars of chemical biology, and at LED3 we have taken it to the next level. ABPP allows the assessment of protein function in live cells and tissues, which means that the activity of a complete protein family can…