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  1. Article ; Online: Mouse Models to Study Peroxisomal Functions and Disorders: Overview, Caveats, and Recommendations.

    Kocherlakota, Sai / Swinkels, Daniëlle / Van Veldhoven, Paul P / Baes, Myriam

    Methods in molecular biology (Clifton, N.J.)

    2023  Volume 2643, Page(s) 469–500

    Abstract: During the last three decades many mouse lines were created or identified that are deficient in one or more peroxisomal functions. Different methodologies were applied to obtain global, hypomorph, cell type selective, inducible, and knockin mice. Whereas ...

    Abstract During the last three decades many mouse lines were created or identified that are deficient in one or more peroxisomal functions. Different methodologies were applied to obtain global, hypomorph, cell type selective, inducible, and knockin mice. Whereas some models closely mimic pathologies in patients, others strongly deviate or no human counterpart has been reported. Often, mice, apparently endowed with a stronger transcriptional adaptation, have to be challenged with dietary additions or restrictions in order to trigger phenotypic changes. Depending on the inactivated peroxisomal protein, several approaches can be taken to validate the loss-of-function. Here, an overview is given of the available mouse models and their most important characteristics.
    MeSH term(s) Animals ; Mice ; Fatty Acids/metabolism ; Peroxisomes/metabolism ; Peroxisomal Disorders/genetics ; Peroxisomal Disorders/metabolism ; Peroxisomal Disorders/pathology
    Chemical Substances Fatty Acids
    Language English
    Publishing date 2023-03-23
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ISSN 1940-6029
    ISSN (online) 1940-6029
    DOI 10.1007/978-1-0716-3048-8_34
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  2. Article ; Online: Identification of Peroxisome-Derived Hydrogen Peroxide-Sensitive Target Proteins Using a YAP1C-Based Genetic Probe.

    Lismont, Celien / Revenco, Iulia / Costa, Cláudio F / Li, Hongli / Hussein, Mohamed A F / Van Veldhoven, Paul P / Derua, Rita / Fransen, Marc

    Methods in molecular biology (Clifton, N.J.)

    2023  Volume 2643, Page(s) 161–181

    Abstract: As the reversible oxidation of protein cysteine thiols is an important mechanism in signal transduction, it is essential to have access to experimental approaches that allow for spatiotemporal indexing of the cellular sulfenome in response to local ... ...

    Abstract As the reversible oxidation of protein cysteine thiols is an important mechanism in signal transduction, it is essential to have access to experimental approaches that allow for spatiotemporal indexing of the cellular sulfenome in response to local changes in H
    MeSH term(s) Animals ; Cysteine/metabolism ; Hydrogen Peroxide/metabolism ; Peroxisomes/metabolism ; Proteins/metabolism ; Sulfhydryl Compounds/metabolism ; Oxidation-Reduction ; Mammals/metabolism
    Chemical Substances Cysteine (K848JZ4886) ; Hydrogen Peroxide (BBX060AN9V) ; Proteins ; Sulfhydryl Compounds
    Language English
    Publishing date 2023-03-23
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ISSN 1940-6029
    ISSN (online) 1940-6029
    DOI 10.1007/978-1-0716-3048-8_12
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  3. Article: Peroxisomal Dysfunction and Oxidative Stress in Neurodegenerative Disease: A Bidirectional Crosstalk.

    Fransen, Marc / Revenco, Iulia / Li, Hongli / Costa, Cláudio F / Lismont, Celien / Van Veldhoven, Paul P

    Advances in experimental medicine and biology

    2021  Volume 1299, Page(s) 19–30

    Abstract: Peroxisomes are multifunctional organelles best known for their role in cellular lipid and hydrogen peroxide metabolism. In this chapter, we review and discuss the diverse functions of this organelle in brain physiology and neurodegeneration, with a ... ...

    Abstract Peroxisomes are multifunctional organelles best known for their role in cellular lipid and hydrogen peroxide metabolism. In this chapter, we review and discuss the diverse functions of this organelle in brain physiology and neurodegeneration, with a particular focus on oxidative stress. We first briefly summarize what is known about the various nexuses among peroxisomes, the central nervous system, oxidative stress, and neurodegenerative disease. Next, we provide a comprehensive overview of the complex interplay among peroxisomes, oxidative stress, and neurodegeneration in patients suffering from primary peroxisomal disorders. Particular examples that are discussed include the prototypic Zellweger spectrum disorders and X-linked adrenoleukodystrophy, the most prevalent peroxisomal disorder. Thereafter, we elaborate on secondary peroxisome dysfunction in more common neurodegenerative disorders, including Alzheimer's disease, Parkinson's disease, and multiple sclerosis. Finally, we highlight some issues and challenges that need to be addressed to progress towards therapies and prevention strategies preserving, normalizing, or improving peroxisome activity in patients suffering from neurodegenerative conditions.
    MeSH term(s) Adrenoleukodystrophy/metabolism ; Adrenoleukodystrophy/pathology ; Alzheimer Disease ; Humans ; Multiple Sclerosis ; Neurodegenerative Diseases/metabolism ; Neurodegenerative Diseases/pathology ; Oxidative Stress ; Parkinson Disease ; Peroxisomes/metabolism ; Peroxisomes/pathology ; Zellweger Syndrome/metabolism ; Zellweger Syndrome/pathology
    Language English
    Publishing date 2021-01-08
    Publishing country United States
    Document type Journal Article ; Review
    ISSN 2214-8019 ; 0065-2598
    ISSN (online) 2214-8019
    ISSN 0065-2598
    DOI 10.1007/978-3-030-60204-8_2
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  4. Article ; Online: The solute carrier SLC25A17 sustains peroxisomal redox homeostasis in diverse mammalian cell lines.

    Costa, Cláudio F / Lismont, Celien / Chornyi, Serhii / Koster, Janet / Li, Hongli / Hussein, Mohamed A F / Van Veldhoven, Paul P / Waterham, Hans R / Fransen, Marc

    Free radical biology & medicine

    2023  Volume 212, Page(s) 241–254

    Abstract: Despite the crucial role of peroxisomes in cellular redox maintenance, little is known about how these organelles transport redox metabolites across their membrane. In this study, we sought to assess potential associations between the cellular redox ... ...

    Abstract Despite the crucial role of peroxisomes in cellular redox maintenance, little is known about how these organelles transport redox metabolites across their membrane. In this study, we sought to assess potential associations between the cellular redox landscape and the human peroxisomal solute carrier SLC25A17, also known as PMP34. This carrier has been reported to function as a counter-exchanger of adenine-containing cofactors such as coenzyme A (CoA), dephospho-CoA, flavin adenine dinucleotide, nicotinamide adenine dinucleotide (NAD
    MeSH term(s) Animals ; Humans ; Mice ; NAD/metabolism ; NADP/metabolism ; Glutathione Disulfide/metabolism ; HeLa Cells ; HEK293 Cells ; Hydrogen Peroxide/metabolism ; Fibroblasts/metabolism ; Peroxisomes/metabolism ; Saccharomyces cerevisiae/genetics ; Saccharomyces cerevisiae/metabolism ; Glutathione/metabolism ; Oxidation-Reduction ; Homeostasis ; Adenine/metabolism ; Mammals/metabolism
    Chemical Substances NAD (0U46U6E8UK) ; NADP (53-59-8) ; Glutathione Disulfide (ULW86O013H) ; Hydrogen Peroxide (BBX060AN9V) ; Glutathione (GAN16C9B8O) ; Adenine (JAC85A2161)
    Language English
    Publishing date 2023-12-29
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 807032-5
    ISSN 1873-4596 ; 0891-5849
    ISSN (online) 1873-4596
    ISSN 0891-5849
    DOI 10.1016/j.freeradbiomed.2023.12.035
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    Zs.A 2109: Show issues Location:
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  5. Article ; Online: The murine retinal pigment epithelium requires peroxisomal β-oxidation to maintain lysosomal function and prevent dedifferentiation.

    Kocherlakota, Sai / Das, Yannick / Swinkels, Daniëlle / Vanmunster, Maarten / Callens, Manon / Vinckier, Stefan / Vaz, Frédéric M / Sinha, Debasish / Van Veldhoven, Paul P / Fransen, Marc / Baes, Myriam

    Proceedings of the National Academy of Sciences of the United States of America

    2023  Volume 120, Issue 43, Page(s) e2301733120

    Abstract: 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 ...

    Abstract 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 multifunctional protein 2 (MFP2) knockout mice impairs the digestive function of lysosomes in the RPE at a very early age, followed by RPE degeneration. This was accompanied by prolonged mammalian target of rapamycin activation, lipid deregulation, and mitochondrial structural anomalies without, however, causing oxidative stress or energy shortage. The RPE degeneration caused secondary photoreceptor death. Notably, the deterioration of the RPE did not occur in an
    MeSH term(s) Mice ; Humans ; Animals ; Retinal Pigment Epithelium/metabolism ; Lysosomes/metabolism ; Phagocytosis/genetics ; Oxidative Stress ; Mice, Knockout ; Mammals
    Language English
    Publishing date 2023-10-20
    Publishing country United States
    Document type Journal Article
    ZDB-ID 209104-5
    ISSN 1091-6490 ; 0027-8424
    ISSN (online) 1091-6490
    ISSN 0027-8424
    DOI 10.1073/pnas.2301733120
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    Zs.A 1148: Show issues Location:
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  6. Article: Peroxisome-Derived Hydrogen Peroxide Modulates the Sulfenylation Profiles of Key Redox Signaling Proteins in Flp-In T-REx 293 Cells.

    Lismont, Celien / Revenco, Iulia / Li, Hongli / Costa, Cláudio F / Lenaerts, Lisa / Hussein, Mohamed A F / De Bie, Jonas / Knoops, Bernard / Van Veldhoven, Paul P / Derua, Rita / Fransen, Marc

    Frontiers in cell and developmental biology

    2022  Volume 10, Page(s) 888873

    Abstract: The involvement of peroxisomes in cellular hydrogen peroxide ( ... ...

    Abstract The involvement of peroxisomes in cellular hydrogen peroxide (H
    Language English
    Publishing date 2022-04-26
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2737824-X
    ISSN 2296-634X
    ISSN 2296-634X
    DOI 10.3389/fcell.2022.888873
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  7. Article ; Online: Editorial Expression of Concern: Glial β-Oxidation regulates Drosophila Energy Metabolism.

    Schulz, Joachim G / Laranjeira, Antonio / Van Huffel, Leen / Gärtner, Annette / Vilain, Sven / Bastianen, Jarl / Van Veldhoven, Paul P / Dotti, Carlos G

    Scientific reports

    2020  Volume 10, Issue 1, Page(s) 13199

    Language English
    Publishing date 2020-07-31
    Publishing country England
    Document type Journal Article ; Expression of Concern
    ZDB-ID 2615211-3
    ISSN 2045-2322 ; 2045-2322
    ISSN (online) 2045-2322
    ISSN 2045-2322
    DOI 10.1038/s41598-020-70053-7
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  8. Article: Slc25a17

    Van Veldhoven, Paul P / de Schryver, Evelyn / Young, Stephen G / Zwijsen, An / Fransen, Marc / Espeel, Marc / Baes, Myriam / Van Ael, Elke

    Frontiers in cell and developmental biology

    2020  Volume 8, Page(s) 144

    Abstract: Mice lacking PMP34, a peroxisomal membrane transporter encoded ... ...

    Abstract Mice lacking PMP34, a peroxisomal membrane transporter encoded by
    Language English
    Publishing date 2020-03-24
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2737824-X
    ISSN 2296-634X
    ISSN 2296-634X
    DOI 10.3389/fcell.2020.00144
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  9. Article ; Online: DHA Shortage Causes the Early Degeneration of Photoreceptors and RPE in Mice With Peroxisomal β-Oxidation Deficiency.

    Swinkels, Daniëlle / Kocherlakota, Sai / Das, Yannick / Dane, Adriaan D / Wever, Eric J M / Vaz, Frédéric M / Bazan, Nicolas G / Van Veldhoven, Paul P / Baes, Myriam

    Investigative ophthalmology & visual science

    2023  Volume 64, Issue 14, Page(s) 10

    Abstract: 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 ... ...

    Abstract 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 retinal decay and cell-autonomous retinal pigment epithelium (RPE) degeneration, accompanied by reduced plasma and retinal DHA levels. In this study, we investigated whether DHA supplementation can rescue the retinal degeneration of Mfp2-/- mice.
    Methods: Mfp2+/- breeding pairs and their offspring were fed a 0.12% DHA or control diet during gestation and lactation and until sacrifice. Offspring were analyzed for retinal function via electroretinograms and for lipid composition of neural retina and plasma with lipidome analysis and gas chromatography, respectively, and histologically using retinal sections and RPE flatmounts at the ages of 4, 8, and 16 weeks.
    Results: DHA supplementation to Mfp2-/- mice restored retinal DHA levels and prevented photoreceptor shortening, death, and impaired functioning until 8 weeks. In addition, rescue of retinal DHA levels temporarily improved the ability of the RPE to phagocytose outer segments and delayed the RPE dedifferentiation. However, despite the initial rescue of retinal integrity, DHA supplementation could not prevent retinal degeneration at 16 weeks.
    Conclusions: We reveal that the shortage of a systemic supply of DHA is pivotal for the early retinal degeneration in Mfp2-/- mice. Furthermore, we report that adequate retinal DHA levels are essential not only for photoreceptors but also for RPE homeostasis.
    MeSH term(s) Humans ; Female ; Animals ; Mice ; Retinal Pigment Epithelium ; Retinal Degeneration ; Docosahexaenoic Acids ; Retina ; Causality
    Chemical Substances Docosahexaenoic Acids (25167-62-8)
    Language English
    Publishing date 2023-11-06
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 391794-0
    ISSN 1552-5783 ; 0146-0404
    ISSN (online) 1552-5783
    ISSN 0146-0404
    DOI 10.1167/iovs.64.14.10
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    Zs.A 45: Show issues Location:
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  10. Article ; Online: Differential activities of peroxisomes along the mouse intestinal epithelium.

    Morvay, Petruta L / Baes, Myriam / Van Veldhoven, Paul P

    Cell biochemistry and function

    2017  Volume 35, Issue 3, Page(s) 144–155

    Abstract: The presence of peroxisomes in mammalian intestine has been revealed formerly by catalase staining combined with electron microscopy. Despite the central role of intestine in lipid uptake and the established importance of peroxisomes in different lipid- ... ...

    Abstract The presence of peroxisomes in mammalian intestine has been revealed formerly by catalase staining combined with electron microscopy. Despite the central role of intestine in lipid uptake and the established importance of peroxisomes in different lipid-related pathways, few data are available on the physiological role of peroxisomes in intestinal metabolism, more specifically, α-, β-oxidation, and etherlipid synthesis. Hence, the peroxisomal compartment was analyzed in more detail in mouse intestine. On the basis of immunohistochemistry, the organelles are mainly confined to the epithelial cells. The expression of the classical peroxisome marker catalase was highest in the proximal part of jejunum and decreased along the tract. PEX14 showed a similar profile, but was still substantial expressed in large intestinal epithelium. Immunoblotting of epithelial cells, isolated from the different segments, showed also such gradient for some enzymes, ie, catalase, ACOX1, and D-specific multifunctional protein 2, and for the ABCD1 transporter, being high in small and low or absent in large intestine. Other peroxisomal enzymes (PHYH, HACL1, and ACAA1), the ABCD2 and ABCD3 transporters, and peroxins PEX13 and PEX14, however, did not follow this pattern, displaying rather constant signals throughout the intestinal epithelium. The small intestine displayed the highest peroxisomal β-oxidation activity and is particularly active on dicarboxylic acids. Etherlipid synthesis was high in the large intestine, and colonic cells had the highest content of plasmalogens. Overall, these data suggest that peroxisomes exert different functions according to the intestinal segment.
    MeSH term(s) Animals ; Catalase/metabolism ; Cells, Cultured ; Fatty Acids/metabolism ; Female ; Intestinal Mucosa/cytology ; Intestinal Mucosa/metabolism ; Male ; Mice ; Mice, Inbred C57BL ; Oxidation-Reduction ; Peroxisomes/metabolism ; Signal Transduction
    Chemical Substances Fatty Acids ; Catalase (EC 1.11.1.6)
    Language English
    Publishing date 2017-04
    Publishing country England
    Document type Journal Article
    ZDB-ID 283643-9
    ISSN 1099-0844 ; 0263-6484
    ISSN (online) 1099-0844
    ISSN 0263-6484
    DOI 10.1002/cbf.3255
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