214 zoekresultaten voor “lysosomal strange disorder” in de Publieke website
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Emily Strange
Co-creatie en multidisciplinair leren over grenzen heen.
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Translational Symmetry Breaking in Holographic Strange Metals
In this thesis, we have used numerical approaches to study the effects of translational symmetry breaking on strange metallic systems as realised by the holographic duality.
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Emily StrangeFaculteit der Wiskunde en Natuurwetenschappen
e.f.strange@cml.leidenuniv.nl | 071 5272727
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Exploring the Versatility of Human β-Glucosidases and Related Glycosylated Metabolites with Novel Chemical Tools
Thesis focused on uncovering the underlying mechanisms of the lysosomal storage disorders Gaucher's disease using chemical tools, biochemical assays and lipidomics.
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Disorder and interactions in high-temperature superconductors
This thesis is devoted to an in-depth examination of the various effects of disorder in the cuprate high-temperature superconductors.
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Nonverbal Learning Disorder (NLD)
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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.
- From basic research to therapy development for neuromuscular disorders
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Exploring strange new worlds with high-dispersion spectroscopy
Until the 1990s, the only known planets were those in our Solar System. Three decades later, several thousand exoplanets have been discovered orbiting stars other than the Sun, and substantial efforts have been made to explore these strange new worlds through spectroscopic analyses of their atmosphe…
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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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chemical biology approach to explore lipid metabolism in neurological disorders
Neurodegenerative diseases pose a large medical and societal challenge. The etiology of these diseases is still poorly understood, which makes drug discovery for these diseases difficult.
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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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Analytical chemistry and biochemistry of glycosphingolipids: new developments and insights
Advanced mass spectrometry of glycosphingolipids takes the central stage in this thesis. Investigations focus on characterization of glycosphingolipid metabolism in health and disease with emphasis to the detection and accurate quantitation of known and so far unknown glycosphingolipids and closely…
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psychosocial development of children with and without Developmental Language Disorder
Emoties en de psychosociale ontwikkeling van kinderen met en zonder Taal Ontwikkelingsstoornis
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Perspectives on Relapse, Recovery, and Emotion Regulation in Eating Disorders
Promotie
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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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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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Applications of AdS/CFT to strongly correlated matter: from numerics to experiments
What physics controls the properties of quantum matter, such as how electrons flow inside high-temperature superconductors? This question has captivated the physics community and industry for decades, in part due to the great technological potential such materials have, but also because they have resisted…
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Transglycosylation of N-acylethanolamines
N-acylethanolamines (NAEs), including N-arachidonoylethanolamine (anandamide, AEA), N-palmitoylethanolamine (PEA) and N-oleoylethanolamine (OEA), are signaling lipids that play an important role in different physiological process.
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Transfer of "goods" from plants to humans: Fundamental and applied biochemical investigations on retaining glycosidases
The studies described in this thesis deal with glycosidases, in particular alpha-galactosidases.
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Design and development of conformational inhibitors and activity-based probes for retaining glycosidases
Glycosidases are essential in fundamental biological processes and are responsible for the degradation of most (oligo)saccharides, glycolipids and glycoproteins.
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The role of zebrafish larvae for studying anxiety-like behaviour
The main aim of my Ph.D. thesis is to further explore the value of using early developing zebrafish larvae (up to 5 dpf) as a model to study anxiety-like behaviour and their pharmacological modulation with drugs.
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Medicijnkandidaat uit Leiden klaar voor laatste klinische onderzoeksfase
Voor veel scheikundigen aan de universiteit is het de ultieme droom: een middel ontwikkelen dat écht bij patiënten terechtkomt. Maar de weg daarnaartoe is lang en onzeker. Nu mag het Leidse middel nizubaglustat door naar de derde fase van klinisch onderzoek. Dat is mogelijk dankzij een investering van…
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Macrophage cell models for the investigation of Gaucher disease
Gaucher disease is a rare metabolic disorder, in which a mutation of the enzyme glucocerebrosidase (GBA1) causes buildup of its substrate glucosylceramide and the deacylated counterpart glucosylsphingosine in lysosomes.
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Nieuwe inzichten in eigenschappen van Conduct Disorder met “beperkte prosociale emoties”
In een recente studie tonen dr. Moji Aghajani en collega’s aan dat jongeren met een ernstige vorm van Conduct Disorder (CD) -met beperkte prosociale emoties- opvallend veel hersencapaciteit nodig hebben om emotionele gezichten te kunnen lezen. Deze effecten werden gevonden in vergelijking met CD-jongeren…
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On electronic signatures of topological superconductivity
Promotor: Prof.dr. C.W.J. Beenakker
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Studies on molecular basics of metabolic syndrome in zebrafish
The research described in this thesis has, using the zebrafish as a model system, shed new light on the intricate relationship between TB and DM2, in particular on the role of leptin, SHP-1 and glucocorticoids.
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Lipidomics study in liver metabolic diseases
The ideas underlying this thesis are that lipidomics may improve the diagnosis of liver metabolic diseases, and can provide further insights into the underlying pathophysiology of these diseases.
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Bioclock: Lichttherapie voor depressie
Optimalisatie, werkingsmechanismen en voorspellers van het resultaat van lichttherapie bij depressie: Een multicenter gerandomiseerde trial
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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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Chemical biology studies on retaining exo-β-glucosidases
The research described in this thesis centered on retaining exo-β-glucosidases implicated in health and disease, in particular the human inherited lysosomal storage disorder, Gaucher disease (GD).
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A computational study of structural and excitonic properties of chlorosomes
The long-held desire - to link structure directly to function and to explain molecular mechanisms based on basic chemical or physical principles - is finally coming closer, satisfying not only our scientific curiosity but also offering new solutions to the many challenges in the field of health, energy…
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Leiden Teachers’ Academy
De Leiden Teachers’ Academy (LTA) wil de kwaliteiten van onze beste docenten zichtbaar maken, verder ontwikkelen en universiteitsbreed benutten. De LTA bestaat uit 26 Teaching Fellows, die alle een passie voor onderwijs hebben.
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Iminosugars as glucosylceramide processing enzymes inhibitors: design, synthesis and evaluation
This Thesis describes the design, synthesis and evaluation as glycoprocessing enzyme inhibitors of focused libraries of iminosugars.
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Marike KooistraFaculteit der Sociale Wetenschappen
m.j.kooistra@fsw.leidenuniv.nl | 071 5272727
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Information Disorder - Publieke Lezing door Eliot Higgins
Lezing
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ENIGMA-Anxiety Werkgroep
Samen met wetenschappers van over de hele wereld streeft de ENIGMA-Anxiety Werkgroep ernaar om meer inzicht te krijgen in de neurobiologische karakteristieken van angststoornissen.
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Lezingen
Tijdens de Leiden Science Family Day worden er vijf lezingen gegeven: twee kinderlezingen en drie lezingen voor volwassenen. Ben jij er bij?
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Bioorthogonal Labeling Tools to Study Pathogenic Intracellular Bacteria
In this thesis, bioorthogonal chemistry is combined with correlative light-electron microscopy to selectively label and study pathogenic intracellular bacteria within the host immune cell.
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Autophagy and Lc3-associated phagocytosis in host defense against Salmonella
Control of infectious diseases poses continuous challenges for human health.
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Selective autophagy in host defense against mycobacterial infection
The effective treatment of tuberculosis (TB) remains a major challenge to global health.
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Exploring chemical space in covalent and competitive glycosidase inhibitor design
Glycoside hydrolases (glycosidases/GHs) are widely abundant enzymes in all kingdoms of life and are important biocatalysts that catalyze the hydrolysis of glycosidic linkages in oligo/polysaccharides, glycoproteins and glycolipids with tremendous efficiency
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Wetenschappelijke publicaties
Een lijst met wetenschappelijke publicaties samengesteld door het team van het TRIXY Expertisecentrum.
- Literatuur
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Empathic or anxious mistakes?
A neurocognitive investigation of the development of social performance monitoring in youth growing up in stressful versus non-stressful environments
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Noonan and LEOPARD syndrome in zebrafish: molecular mechanisms and cardiac development
Promotor: Prof.dr. J. den Hertog
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Podcast: Een leven met Bipolaire Stoornis
We praten met Justin, die ruim 14 jaar geleden gediagnostiseerd werd met Bipolaire Stoornis type 1. Justin is erg openhartig en vertelt over zijn eerste manische episode op 28 jarige leeftijd, en hoe hij heeft geleerd om een stabiel leven te kunnen leiden. Met Justin gaat het goed, en dat heeft hij…
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Seksuele responsen zijn aan én af te leren
Ongewenste associaties met seks kunnen afgeleerd worden, maar terugkeren als de omstandigheden veranderen. Daarom moet het afleren in diverse situaties plaatsvinden. Het geneesmiddel D-cycloserine biedt mogelijk uitkomst. Dat ontdekte psychologe Mirte Brom.
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Visualizing strongly-correlated electrons with a novel scanning tunneling microscope
Materials with strongly correlated electrons show some of the most mysterious and exotic phases of quantum matter, such as unconventional superconductivity, quantum criticality and strange metal phase.
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Onderwijsprijs
Elk jaar reikt het Leids Universitair Studentenplatform (LUS) de Leidse Onderwijsprijs uit.