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North Sea seabirds and marine mammals: pathology and ecotoxicology = De zeevogels en zeezoogdieren van de Noordzee: pathologie en ecotoxicologie Instituut voor Natuur- en Bosonderzoek
Benefits and Caveats in the Use of Retinal Pigment Epithelium-Specific Cre Mice KU Leuven
The retinal pigment epithelium (RPE) is an important monolayer of cells present in the outer retina, forming a major part of the blood-retina barrier (BRB). It performs many tasks essential for the maintenance of retinal integrity and function. With increasing knowledge of the retina, it is becoming clear that both common retinal disorders, like age-related macular degeneration, and rare genetic disorders originate in the RPE. This calls for a ...
Peroxisomal β-oxidation is crucial for the degradation of photoreceptor outer segments by the retinal pigment epithelium. KU Leuven
Retinal pigment epithelium (RPE) cells have to phagocytose shed photoreceptor outer segments (POS) on a daily basis over the lifetime of an organism, but the mechanisms involved in the digestion and recycling of POS lipids are poorly understood. Although it was frequently assumed that peroxisomes may play an essential role, this was never investigated. Since the RPE cells need to digest large amounts of VLC-PUFAs (very long chain polyunsaturated ...
DHA Shortage Causes the Early Degeneration of Photoreceptors and RPE in Mice With Peroxisomal β-Oxidation Deficiency KU Leuven
PURPOSE: Patients deficient in peroxisomal β-oxidation, which is essential for the synthesis of docosahexaenoic acid (DHA, C22:6n-3) and breakdown of very-long-chain polyunsaturated fatty acids (VLC-PUFAs), both important components of photoreceptor outer segments, develop retinopathy present with retinopathy. The representative mouse model lacking the central enzyme of this pathway, multifunctional protein 2 (Mfp2-/-), also show early-onset ...
The murine retinal pigment epithelium requires peroxisomal β-oxidation to maintain lysosomal function and prevent dedifferentiation KU Leuven
Retinal pigment epithelium (RPE) cells have to phagocytose shed photoreceptor outer segments (POS) on a daily basis over the lifetime of an organism, but the mechanisms involved in the digestion and recycling of POS lipids are poorly understood. Although it was frequently assumed that peroxisomes may play an essential role, this was never investigated. Here, we show that global as well as RPE-selective loss of peroxisomal β-oxidation in ...
The essential role of docosahexaenoic acid and its derivatives for retinal integrity KU Leuven
The fatty acid composition of photoreceptor outer segment (POS) phospholipids diverges from other membranes, being highly enriched in polyunsaturated fatty acids (PUFAs). The most abundant PUFA is docosahexaenoic acid (DHA, C22:6n-3), an omega-3 PUFA that amounts to over 50% of the POS phospholipid fatty acid side chains. Interestingly, DHA is the precursor of other bioactive lipids such as elongated PUFAs and oxygenated derivatives. In this ...
Enhanced Levels of Peroxisome-Derived H2O2 Do Not Induce Pexophagy but Impair Autophagic Flux in HEK-293 and HeLa cells KU Leuven
Peroxisomes are functionally specialized organelles that harbor multiple hydrogen peroxide (H2O2)-producing and -degrading enzymes. Given that this oxidant functions as a major redox signaling agent, peroxisomes have the intrinsic ability to mediate and modulate H2O2-driven pro-cesses, including autophagy. However, it remains unclear whether changes in peroxisomal H2O2 (po-H2O2) emission impact the autophagic process and to which extent ...
Unexpected failure of rod bipolar cell targeting using L7Cre-2 mice KU Leuven
Utilizing cell type-specific knockout mice has been an excellent tool for decades not only to explore the role of a gene in a specific cell, but also to unravel the underlying mechanism in diseases. To investigate the mechanistic association between dysfunction of the peroxisomal protein multifunctional protein 2 (MFP2) and retinopathy, we generated and phenotyped multiple transgenic mouse models with global or cell type-specific MFP2 deletion. ...
Normal plasmalogen levels are maintained in tissues from mice with hepatocyte-specific deletion in peroxin 5 KU Leuven
On the basis of findings that cultured rat hepatocytes secrete lipoprotein with a high plasmalogen content and the occurrence of this lipid in human serum, it has been suggested that hepatocytes play a role in the supply of plasmalogens to tissues. We tested this hypothesis in a mouse with a hepatocyte-specific defect in peroxisomes, an organelle essentially required for plasmalogen biosynthesis. We analyzed plasmalogens in lipid extracts of ...