777 zoekresultaten voor “lysosomal state disorders” in de Publieke website
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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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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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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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Staatscommissies/State committees
In this project of the Center for Public Values & Ethics (CPVE) ongoing research is conducted on the history of advice to Dutch central government by so-called State committees from 1814 to 2014. State committees have been installed by Dutch central government since 1814 to provide expert advice, research…
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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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Gibbs States in Statistical Mechanics and Dynamical Systems
This dissertation is organised into three parts, each addressing a distinct research area related to Gibbs measures.
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Steady-State Analysis of Large Scale Systems
Promotor: W.Th.F. den Hollander Co-Promotor: F.M. Spieksma
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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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Internationaal boekproject Scarcity and the State
Onder de gezamenlijke titel ‘Scarcity and the State’ verschenen onlangs twee Engelstalige boeken over de verdeling van schaarse publieke rechten (Intersentia 2016). Paul Adriaanse (UL), Frank van Ommeren (VU), Willemien den Ouden (UL) en Johan Wolswinkel (Tilburg University) voerden de redactie. Aan…
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Revealing private interests of non-state actor coalitions in negotiating access and benefit sharing
Dit artikel onderzoekt hoe tijdelijke coalities van niet-statelijke actoren deelnemen aan internationale onderhandelingen over milieuvraagstukken.
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Lezingreeks: Humanity in the Automated State
De lezingreeks Humanity in the Automated State Jaar 2025/2026 onderzoekt hoe AI en geautomatiseerde systemen de overheid en publieke administratie transformeren en wat het betekent om mens te zijn binnen deze gedigitaliseerde instituties.
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Establishing State Responsibility in the Absence of Effective Government
Op 16 juni 2020 verdedigde Andrea Varga haar proefschrift 'Establishing State Responsibility in the Absence of Effective Government'. Het promotieonderzoek is begeleid door promotoren prof. dr. N.J. Schrijver en prof. dr. F. Baetens (University of Oslo).
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State-resolved studies of CO2 gas-surface reactions
The adsorption of D2 and CO2 on catalyst surfaces is studied using a molecular beam in ultra-high vacuum.
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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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Nieuw handboek “EU State Aids”
Het doet het Europa Instituut genoegen aan te kondigen dat op 21 november 2016 de 5e druk is verschenen van het handboek “EU State Aids” (31 hoofdstukken, 1500 pagina’s).
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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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Quantum dots in microcavities: From single spins to engineered quantum states of light
A single self-assembled semiconductor quantum dot in a high-finesse optical microcavity - the subject of this thesis - is an interesting quantum-mechanical system for future quantum applications. For instance, this system allows trapping of an extra electron and thus can serve as a spin quantum memory,…
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Public Health and the American State
Dit boek onderzoekt hoe zorgen over de volksgezondheid en politieke agenda’s elkaar in de VS de afgelopen eeuw hebben beïnvloed.
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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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Strategies for braiding and ground state preparation in digital quantum hardware
With the help of quantum mechanics, digital quantum hardware may be able to tackle some of the problems that are too difficult for ordinary computers. But despite these expectations and the ongoing effort of the research community, reliable quantum computers are not yet realized in a lab setting.
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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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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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On electronic signatures of topological superconductivity
Promotor: Prof.dr. C.W.J. Beenakker
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Domestic Courts in Investor-State Arbitration: Partners, Suspects, Competitors
Op 27 juni 2019 verdedigde Vid Prislan zijn proefschrift 'Domestic Courts in Investor-State Arbitration: Partners, Suspects, Competitors'. Het promotieonderzoek is begeleid door promotor prof. dr. N.J. Schrijver.
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States in shock: De aanpassingscapaciteiten van staatsadministraties bij grensoverschrijdende crises
Deze studie onderzoekt hoe Europese staatsadministraties de afgelopen 30 jaar hebben gereageerd op grensoverschrijdende crises en in hoeverre zij succesvol zijn aangepast.
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State Silence and the International Law of Cyberspace
This article offers an inaugural assessment of how silences implicate international law-making in cyberspace through descriptive and normative lenses.
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Let Them Roar: Small States as Cyber Norm Entrepreneurs
Sinds de United Nations Group of Governmental Experts on the Developments in the Field of Information and Telecommunications in the Context of International Security (UN GGE) elf aanbevelingen heeft gedaan over verantwoordelijk staatsgedrag omtrent cyberspace is er een discussie gaande over internationale…
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Catecholamine function, brain state dynamics, and human cognition
Het onderzoek in dit proefschrift kijkt naar de rol van het locus ceuruleus - noradrenalinesysteem in verscheidene aspecten van menselijke cognitie, en de modulatie van de staat van het brein.
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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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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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Luc Verhey wordt lid van de Raad van State
Luc Verheij hoogleraar Staats en bestuursrecht (Kircheiner-leerstoel) in Leiden wordt lid van de Raad van State. Sinds 2011 is Verheij al staatsraad in de Afdeling advisering. Deze rol blijft hij vervullen en wordt daarnaast ook lid van deze Raad.
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Foreign intelligence in the digital age. Navigating a state of 'unpeace'.
Het The Hague Program for Cyber Norms, een onderzoeksprogramma van het Institute of Security and Global Affairs, heeft zijn eerste beleidsnota gepubliceerd. Hierin onderzoeken Dennis Broeders, Sergei Boeke en Ilina Georgieva de rol van inlichtingendiensten in cyberspace en de (on)mogelijkheden van adequate…
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(Non)recognition of legal identity in aspirant states: evidence from Abkhazia, South Ossetia and Transnistria
Ramesh Ganohariti onderzoekt de juridische identiteit in drie post-Sovjet aspirant-lidstaten en schetst in dit artikel vier veelvoorkomende scenario's.
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Rebel Legal Order, Governance and Legitimacy: Examining the Islamic State and the Taliban Insurgency
Dit artikel onderzoekt hoe ISIS en de Taliban steun hebben gekregen door hun parallelle rechtssystemen.
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Freedom of Overflight: A Study of Coastal State Jurisdiction in International Airspace
Op 10 juni 2021 verdedigde Merinda Stewart haar proefschrift 'Freedom of Overflight: A Study of Coastal State Jurisdiction in International Airspace'. Het promotieonderzoek is begeleid door promotoren prof.dr. P.M.J. Mendes de Leon en prof.dr. J.J. Rijpma.
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Into the ether or the state? Legibility theory and the cryptocurrency markets
In dit artikel presenteren Kellerman en Seddon een nieuw conceptueel kader waarin het concept van marktleesbaarheid centraal staat.
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Borders in Dispute: The Construction of State and Nation in International Diplomacy
How are borders drawn? More specifically, how do diplomatic actors manage the implications of changes in state sovereignty for international borders?
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informality of the international cybersecurity regime: Geopolitics, non-state actors and diplomacy
De auteurs analyseren twee belangrijke factoren die informaliteit verdiepen, namelijk multipolaire geopolitiek en de opkomst van niet-statelijke actoren.
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Small-State Influence in EU Security Governance: Unveiling Latvian Lobbying Against Disinformation
In dit artikel onderzoekt Sophie Vériter hoe een kleine staat het veiligheidsbestuur van de EU beïnvloedt.
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Principles of Evidence in Investor-State Arbitration, Burden, Standards, Presumptions & Inferences
Op 28 februari 2019 verdedigde Kabir Duggal zijn proefschrift 'Principles of Evidence in Investor-State Arbitration, Burden, Standards, Presumptions & Inferences'. Het promotieonderzoek is begeleid door promotor prof. dr. E. De Brabandere.
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Unpacking the effects of burdensome state actions on citizens' policy perceptions
In dit artikel onderzochten Martin Sievert en Jonas Bruder of en hoe administratieve lasten van invloed zijn op de perceptie van burgers van het welzijnsbeleid en hun houding tegenover uitkeringsgerechtigden.
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Nieuw handboek “EU State Aids”
Het doet het Europa Instituut genoegen aan te kondigen dat deze week de 5e druk is verschenen van het handboek “EU State Aids” (31 hoofdstukken, 1500 pagina’s).
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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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Evolution and development of flowers, fruits and inflorescences of Phalaenopsis and other orchid species
The study of orchid flowers, fruits, and inflorescences is crucial due to the remarkable diversity of orchid species and their unique adaptations to pollinators and seed dispersers. However, our understanding of the evolution and development of these organs within the orchid family remains limited.
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‘Raad van State is het geweten van de overheid’
Al zijn de adviezen van de Raad van State niet bindend, ze zijn zeker gezaghebbend. Dat zei Piet Hein Donner of 4 februari tijdens een drukbezochte lezing aan de Rechtenfaculteit.