A5080893941- Lipid metabolism and biosynthesis
- Peroxisome Proliferator-Activated Receptors
- Plant biochemistry and biosynthesis
- Skin and Cellular Biology Research
- Enzyme Catalysis and Immobilization
- Metabolism and Genetic Disorders
- Photosynthetic Processes and Mechanisms
- Retinal Development and Disorders
- Neurological diseases and metabolism
- Endoplasmic Reticulum Stress and Disease
- RNA regulation and disease
- Urban Transport Systems Analysis
- Parkinson's Disease Mechanisms and Treatments
- Cholesterol and Lipid Metabolism
- Biochemical and Molecular Research
- Toxin Mechanisms and Immunotoxins
- RNA modifications and cancer
- Fermentation and Sensory Analysis
- Phytase and its Applications
- Yeasts and Rust Fungi Studies
- Plant Molecular Biology Research
- Ubiquitin and proteasome pathways
- Nematode management and characterization studies
- Genetics and Neurodevelopmental Disorders
- Systemic Sclerosis and Related Diseases
Radboud University Nijmegen
2020-2025
Radboud University Medical Center
2021-2025
Radboud Institute for Molecular Life Sciences
2021-2025
Université Claude Bernard Lyon 1
2016-2021
Centre National de la Recherche Scientifique
2016-2021
Inserm
2016-2021
École Normale Supérieure de Lyon
2016-2021
Structure Fédérative de Recherche Biosciences
2018-2021
Lyon College
2020
King's College London
2006-2013
Alpha-synuclein is a small cytosolic protein involved in the pathogenesis of Parkinson's disease and other neurodegenerative disorders. Recent studies suggested lipid-related function for this brain-enriched protein. Since brain carries high level docosahexaenoic acid (DHA) since extent alpha-synuclein gene expression increases response to DHA intake, we have investigated interaction with essential omega-3 fatty acid. We show that allows be present soluble rather than micellar form. Upon...
Increasing evidence suggests that dysregulation of lipid metabolism is associated with neurodegeneration in retinal diseases such as age-related macular degeneration and brain disorders Alzheimer’s Parkinson’s diseases. Lipid storage organelles (lipid droplets, LDs), accumulate many cell types response to stress, it now clear LDs function not only stores but also dynamic regulators the stress response. However, whether these are always protective or can be deleterious unknown. Here, we...
Parkinson’s disease (PD) is a neurodegenerative disorder characterized by alpha-synuclein (αSyn) aggregation and associated with abnormalities in lipid metabolism. The accumulation of lipids cytoplasmic organelles called droplets (LDs) was observed cellular models PD. To investigate the pathophysiological consequences interactions between αSyn proteins that regulate homeostasis LDs, we used transgenic Drosophila model PD, which human specifically expressed photoreceptor neurons. We first...
ABSTRACT Roots located in the upper soil layers are prone to experiencing high temperatures. Despite their importance for water and nutrient absorption, little is known about effect of temperature on root hairs. Here, we found that exposure Arabidopsis thaliana seedlings long‐term mild heat suppressed hair initiation. Epidermal patterning non‐hair cells was maintained, as observed with GL2 ‐ CPC ‐based marker genes, suppression independent activity its upstream regulators. Instead,...
Phytol, a branched-chain fatty alcohol, is the naturally occurring precursor of phytanic and pristanic acid, acids that are both ligands for nuclear hormone receptor peroxisome proliferator-activated alpha (PPARalpha). To investigate metabolism phytol role PPARalpha in its regulation, wild-type knockout (PPARalpha-/-) mice were fed phytol-enriched diet or, comparison, enriched with Wy-14,643, synthetic agonist. After diet, could only be detected small intestine, site uptake, liver. Upon...
The biochemical hallmark of adult Refsum disease (ARD) is an isolated deficiency in the breakdown phytanic acid. This usually results from a PHYH gene defect, although some cases have been found to carry PEX7 defect. We describe phenotype such patient, indistinguishable that classic ARD. Hence, we propose subdivision ARD into type 1 and 2, depending on which defective.
Phytol is a naturally occurring precursor of phytanic acid. The last step in the conversion phytol to phytanoyl‐CoA reduction phytenoyl‐CoA mediated by an, as yet, unidentified enzyme. A candidate for this reaction previously described peroxisomal trans ‐2‐enoyl‐CoA reductase (TER). To investigate this, human TER was expressed E. coli an MBP‐fusion protein. purified recombinant protein shown have high activity towards ‐phytenoyl‐CoA, but not β‐oxidation intermediates C24:1‐CoA and...
Phytol is a branched-chain fatty alcohol that naturally occurring precursor of phytanic acid, acid involved in the pathogenesis Refsum disease. The conversion phytol into generally believed to take place via three enzymatic steps involve 1) oxidation its aldehyde, 2) further phytenic and 3) reduction double bond at 2,3 position, yielding acid. Our recent investigations this mechanism have elucidated leading production, but final step pathway has not been investigated so far. In study, we...
Fatty aldehyde dehydrogenase (FALDH) is an NAD+-dependent oxidoreductase involved in the metabolism of fatty alcohols. Enzyme activity has been implicated pathology diabetes and cancer. Mutations human gene inactivate enzyme cause accumulation alcohols Sjögren-Larsson syndrome, a neurological disorder resulting physical mental handicaps. Microsomal FALDH was expressed E. coli purified. Using vitro assay optimum pH ∼9.5 temperature ∼35°C were determined. Medium- long-chain aldehydes converted...
Sjogren–Larsson syndrome (SLS) is a metabolic disorder characterized by an accumulation of long-chain fatty alcohols in plasma (1)(2). Studies Rizzo and coworkers (3)(4)(5) have identified the enzyme that deficient SLS as aldehyde dehydrogenase (FALDH; EC 1.2.1.3), which encoded ALDH10 gene. FALDH part microsomal alcohol NAD+-oxidoreductase complex, functions conversion into acids (3). A deficiency leads to (4)(5). Interestingly, also plays role degradation leukotriene B4 (6). In our...
Nervous system function requires that neurons within neural circuits are connected together precisely. These connections form during the process of axon guidance whereby each neuron extends an migrates, often large distances, through a complex environment to reach its synaptic target. This task can be simplified by utilising intermediate targets divide route into smaller sections. axons adapt their behaviour as they migrate towards and away from targets. In central nervous midline acts...
Abstract Parkinson’s disease (PD) is a neurodegenerative disorder characterized by alpha-synuclein (αSyn) aggregation and associated with abnormalities in lipid metabolism. The accumulation of lipids cytoplasmic organelles called droplets (LDs) was observed cellular models PD. To investigate the pathophysiological consequences interactions between αSyn proteins that regulate homeostasis LDs, we used transgenic Drosophila model PD, which human specifically expressed photoreceptor neurons. We...
Abstract Increasing evidence suggests that dysregulation of lipid metabolism is associated with neurodegeneration in retinal diseases such as age-related macular degeneration and brain disorders Alzheimer’s Parkinson’s diseases. Lipid storage organelles (lipid droplets, LDs), accumulate many cell types response to stress, it now clear LDs function not only stores but also dynamic regulators the stress response. However, whether these LD functions are always protective or can be deleterious...