838 search results for “lysosomal storage disorder” 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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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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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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Martijn van der Lienden
Science
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Lindsey Lelieveld
Science
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Applications for activity-based probes in biomedical research on glycosidases
Lysosomal glycosidases are acid hydrolases that fragment glycoconjugates in lysosomes.
- Chi-Lin Kuo
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Chemical biology of sphingolipids: fundamental studies and clinical applications
Overkleeft
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Azafaros is valorizing a library of compounds
The company Azafaros was established in 2018 to translate innovative science from Leiden University and Amsterdam University Medical Center into novel disease-modifying treatment modalities for rare genetic metabolic disorders such as lysosomal storage diseases.
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PhD candidate , Lysosomal delivery of intracellular pathogens
Science, Institute of Biology Leiden (IBL)
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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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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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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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Gorlaeus Bicycle Storage
The sustainable demolition of the Gorlaeus high-rise of the Faculty of Science is now complete. This demolition is part of the redevelopment of the faculty. A part of the steel construction will form the basis of a new green bike park.
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Gorlaeus Bicycle Storage
A part of the steel construction of the former Gorlaeus high-rise will form the basis of a new green bike park which is part of the redevelopment of the Faculty of Science.
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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…
- Scheduled maintenance storage Infrastructure
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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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Medical Biochemistry
Medical Biochemistry is a section of the Leiden Institute of Chemistry at Leiden University. The section is headed by Prof. Dr. Hans Aerts.
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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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Image analysis & storage
Obtaining imaging data is nowadays only the start of your project. Once the data has been acquired there are many things that still need to be done.
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Translating science into treatments of rare metabolic disorders
Leiden biotech startup Azafaros has successfully completed a funding round, raising 25 million euros of investments for developing treatments of rare metabolic disorders. The company holds exclusive license to a library of novel patented compounds discovered by experts from Leiden University.
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Nonverbal Learning Disorder (NLD)
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The Leiden Family Lab study on Social Anxiety Disorder
Social anxiety runs in families, but the neurobiology underlying this genetic vulnerability is until now largely unknown. The unique Leiden Family Lab study on Social Anxiety Disorder (LFLSAD) aims to broaden our knowledge with respect to this genetic susceptibility.
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Hydrogen: Production and Storage of Hydrogen
The Hydrogen network brings together leading researchers from different disciplines and sectors with a combined expertise that maximises the chance of achieving scientific breakthroughs in production and storage of hydrogen, while guaranteeing the successful training of a new generation of scientists…
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OPENup: Optimizing exposure therapy for Posttraumatic Stress disorder
Although exposure therapy is the gold-standard treatment of posttraumatic stress-disorder (PTSD), only half of the patients adequately benefit from it. Based on recent insights into the mechanisms of extinction, this project examines strategies and techniques to improve exposure treatment outcome in…
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Topic: Stress and stress-related disorders
The precise psychobiological mechanisms leading to chronic physiological stress responses have not been sufficiently explained, although stress is a major risk factor for disease and early death. A problem for conventional stress theory is that most of these responses seem to occur in situations without…
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Predicting alcohol use disorder through machine learning
How to come to valid risk stratification of alcohol use disorder?
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Project 2: From Disorder to Order
One of the current research projects funded by the profile area Political Legitimacy is the research group From Disorder to Order: Conflict and the Resources of Legitimacy.
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scanning tunneling microscopy on conventional, unconventional and disordered superconductors
In this thesis we use Josephson and noise scanning tunneling microscopy for the study of conventional, unconventional (iron-based) and disordered superconductors. On the one hand, Josephson scanning tunneling microscopy allows us to directly visualize the superfluid density with high spatial resolut…
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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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Chemical biology of glucosylceramide metabolism: fundamental studies and clinical applications for Gaucher disease
How can we develop new chemical biology tools and approaches to understand and interfere with glucosylceramide metabolism in relation to Gaucher disease?
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Profiling Endophenotypes in Social Anxiety Disorder – a family study
The key question addressed in this family study is whether the psychophysiological and neurocognitive abnormalities often reported in SAD patients are heritable and can thus be found in family members of SAD patients as well. Determination of heritability of these deficiencies is essential for endophenotyping.…
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patients with Borderline Personality Disorder and patients with Attention Deficit Hyperactivity Disorder
How does stress affect different components of behavioral impulsivity and aggression in patients with Borderline Personality Disorder compared to clinical controls (patients with Attention Deficit Hyperactivity Disorder) and healthy controls?
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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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Emotion regulation in children and adolescents with an Autism Spectrum Disorder
Why do children with an Autism Spectrum Disorder (ASD) have difficulties with acquiring emotion regulation skills; and how is this related to their social impairments and comorbid psychopathology?
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neural correlates of Emotional Working Memory in Borderline Personality Disorder
How do dissociative states affect the ability to suppress emotional distraction (in the context of a working memory task) in patients with Borderline Personality Disorder?
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training on emotion regulation capacities in Borderline Personality Disorder
Does an Emotional Working Memory Training show a beneficial transfer effect on emotion regulation (reappraisal) capacities in patients with Borderline Personality Disorder?
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Improved storage stability and immunogenicity of hepatitis B vaccine after spray-freeze drying in presence of sugars
The current hepatitis B vaccines need to be stored and transported under refrigerated conditions (2-8 degrees C). This dependence on a cold-chain is highly challenging in areas where hepatitis B virus infections are endemic.
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Social Feedback and Emotion Regulation in Borderline Personality Disorder: An fMRI study
What is the role of emotion regulation in coping with interpersonal feedback?
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psychosocial development of children with and without Developmental Language Disorder
Dissertation
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Criteria for recovery from eating disorder patients: a comparison between therapists, patients and a healthy control group
Which criteria do ED patients and therapists evaluate as most relevant for recovery? Do patients and therapists differ in their evaluations? Do eating disorder patients differ from a healthy control group in their scores on the Recovery Scale?
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Projects
Research projects
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Podcast: A life with Bipolar Disorder
We talk to Justin Sheehan, who was diagnosed with Bipolar Disorder type 1 over 14 years ago. Justin is very candid and talks about his first manic episode at the age of 28, and how he learned to live a stable life. Justin is doing well, and he owes this in particular to his own perseverance. What do…
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Philip Spinhoven
Faculteit der Sociale Wetenschappen
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Jacqueline Guicherit-Dicke
Faculteit der Sociale Wetenschappen
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People with an eating disorder benefit from eHealth
It often takes a long time for people with an eating disorder to seek help and receive treatment. eHealth could be a solution, PhD candidate Pieter Rohrbach has discovered.
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Breakthrough by Leiden researchers in Pompe disease
Researchers at Leiden University have made a breakthrough in the study of the hereditary Pompe disease. Together with colleagues in York, they have developed a molecule that binds to the enzyme that is key to the progress of the disease. The findings have been published in ACS Central Science.
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Podcast: Social Anxiety Disorder
Have you ever experienced the feeling of awkwardness when attending a party where you didn’t know anybody? Ever felt shy at a party within the first few minutes? While this feeling is labelled loosely as feeling socially anxious, social anxiety disorder goes to a much further extent.
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LED3 Drug Discovery Case Studies
To get a feeling of how we operate at LED3 when it comes to Early Drug Discovery, please browse through our case studies. When you select a case study you’ll find relevant contacts.