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  1. Article ; Online: Benefits and Caveats in the Use of Retinal Pigment Epithelium-Specific Cre Mice.

    Kocherlakota, Sai / Baes, Myriam

    International journal of molecular sciences

    2024  Volume 25, Issue 2

    Abstract: 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 ... ...

    Abstract 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 better understanding of the functions of various proteins within the RPE. In this regard, mice enabling an RPE-specific gene deletion are a powerful tool to study the role of a particular protein within the RPE cells in their native environment, simultaneously negating any potential influences of systemic changes. Moreover, since RPE cells interact closely with adjacent photoreceptors, these mice also provide an excellent avenue to study the importance of a particular gene function within the RPE to the retina as a whole. In this review, we outline and compare the features of various Cre mice created for this purpose, which allow for inducible or non-inducible RPE-specific knockout of a gene of interest. We summarize the various benefits and caveats involved in the use of such mouse lines, allowing researchers to make a well-informed decision on the choice of Cre mouse to use in relation to their research needs.
    MeSH term(s) Mice ; Animals ; Retinal Pigment Epithelium/metabolism ; Retina ; Photoreceptor Cells ; Macular Degeneration/genetics ; Macular Degeneration/metabolism ; Proteins/metabolism ; Mice, Knockout
    Chemical Substances Proteins
    Language English
    Publishing date 2024-01-20
    Publishing country Switzerland
    Document type Journal Article ; Review
    ZDB-ID 2019364-6
    ISSN 1422-0067 ; 1422-0067 ; 1661-6596
    ISSN (online) 1422-0067
    ISSN 1422-0067 ; 1661-6596
    DOI 10.3390/ijms25021293
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: The essential role of docosahexaenoic acid and its derivatives for retinal integrity.

    Swinkels, Daniëlle / Baes, Myriam

    Pharmacology & therapeutics

    2023  Volume 247, Page(s) 108440

    Abstract: 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 ... ...

    Abstract 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 review, we present the current view on metabolism, trafficking and function of DHA and very long chain polyunsaturated fatty acids (VLC-PUFAs) in the retina. New insights on pathological features generated from PUFA deficient mouse models with enzyme or transporter defects and corresponding patients are discussed. Not only the neural retina, but also abnormalities in the retinal pigment epithelium are considered. Furthermore, the potential involvement of PUFAs in more common retinal degeneration diseases such as diabetic retinopathy, retinitis pigmentosa and age-related macular degeneration are evaluated. Supplementation treatment strategies and their outcome are summarized.
    MeSH term(s) Mice ; Animals ; Docosahexaenoic Acids/pharmacology ; Docosahexaenoic Acids/therapeutic use ; Docosahexaenoic Acids/analysis ; Retina/metabolism ; Fatty Acids, Omega-3 ; Fatty Acids, Unsaturated/metabolism ; Fatty Acids/metabolism
    Chemical Substances Docosahexaenoic Acids (25167-62-8) ; Fatty Acids, Omega-3 ; Fatty Acids, Unsaturated ; Fatty Acids
    Language English
    Publishing date 2023-05-16
    Publishing country England
    Document type Journal Article ; Review
    ZDB-ID 194735-7
    ISSN 1879-016X ; 0163-7258
    ISSN (online) 1879-016X
    ISSN 0163-7258
    DOI 10.1016/j.pharmthera.2023.108440
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    In stock of ZB MED Cologne/Königswinter

    Zs.A 1183: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
    Jg. 1995 - 2021: Lesesall (1.OG)
    ab Jg. 2022: Lesesaal (EG)

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  3. Article ; Online: Unexpected failure of rod bipolar cell targeting using L7Cre-2 mice.

    Peperstraete, Kaat / Baes, Myriam / Swinkels, Daniëlle

    Experimental eye research

    2023  Volume 228, Page(s) 109406

    Abstract: 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 ... ...

    Abstract 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. These studies pointed to a potential role of MFP2 specifically in rod bipolar cells. To explore this, we aimed to create rod bipolar cell specific knockout mice of Mfp2 by crossing Mfp2
    MeSH term(s) Mice ; Animals ; Mice, Transgenic ; Retinal Bipolar Cells ; Purkinje Cells ; Mice, Knockout
    Language English
    Publishing date 2023-02-04
    Publishing country England
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 80122-7
    ISSN 1096-0007 ; 0014-4835
    ISSN (online) 1096-0007
    ISSN 0014-4835
    DOI 10.1016/j.exer.2023.109406
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 163: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
    Jg. 1995 - 2021: Lesesall (1.OG)
    ab Jg. 2022: Lesesaal (EG)

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  4. Article: Developmental and Degenerative Cerebellar Pathologies in Peroxisomal β-Oxidation Deficiency.

    De Munter, Stephanie / Baes, Myriam

    Advances in experimental medicine and biology

    2021  Volume 1299, Page(s) 105–115

    Abstract: The integrity of the cerebellum is exquisitely dependent on peroxisomal β-oxidation metabolism. Patients with peroxisomal β-oxidation defects commonly develop malformation, leukodystrophy, and/or atrophy of the cerebellum depending on the gene defect and ...

    Abstract The integrity of the cerebellum is exquisitely dependent on peroxisomal β-oxidation metabolism. Patients with peroxisomal β-oxidation defects commonly develop malformation, leukodystrophy, and/or atrophy of the cerebellum depending on the gene defect and on the severity of the mutation. By analyzing mouse models lacking the central peroxisomal β-oxidation enzyme, multifunctional protein-2 (MFP2), either globally or in selected cell types, insights into the pathomechanisms could be obtained. All mouse models developed ataxia, but the onset was earlier in global and neural-selective (Nestin) Mfp2
    MeSH term(s) Animals ; Axons/metabolism ; Axons/pathology ; Cerebellum/metabolism ; Cerebellum/pathology ; Humans ; Oxidation-Reduction ; Peroxisomes/metabolism ; Purkinje Cells/metabolism ; Purkinje Cells/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_9
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. 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
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article: Enhanced Levels of Peroxisome-Derived H

    Li, Hongli / Lismont, Celien / Costa, Cláudio F / Hussein, Mohamed A F / Baes, Myriam / Fransen, Marc

    Antioxidants (Basel, Switzerland)

    2023  Volume 12, Issue 3

    Abstract: Peroxisomes are functionally specialized organelles that harbor multiple hydrogen peroxide ( ... ...

    Abstract Peroxisomes are functionally specialized organelles that harbor multiple hydrogen peroxide (H
    Language English
    Publishing date 2023-03-02
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2704216-9
    ISSN 2076-3921
    ISSN 2076-3921
    DOI 10.3390/antiox12030613
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  7. Article: Peroxisomal Disorders and Retinal Degeneration.

    Das, Yannick / Baes, Myriam

    Advances in experimental medicine and biology

    2019  Volume 1185, Page(s) 317–321

    Abstract: Peroxisomal disorders are a group of inherited metabolic diseases, which can be incompatible with life in the postnatal period or allow survival into adulthood. Retinopathy is a recurrent feature in both the severely and mildly affected patients, which ... ...

    Abstract Peroxisomal disorders are a group of inherited metabolic diseases, which can be incompatible with life in the postnatal period or allow survival into adulthood. Retinopathy is a recurrent feature in both the severely and mildly affected patients, which can be accompanied with other ophthalmological pathologies. Thanks to next-generation sequencing, patients originally identified with other inherited blinding diseases were reclassified as suffering from peroxisomal disorders. In addition, new peroxisomal gene defects or disease presentations exhibiting retinal degeneration were recently identified. The pathogenic mechanisms underlying retinopathy in peroxisomal disorders remain unresolved.
    MeSH term(s) Humans ; Peroxisomal Disorders/complications ; Peroxisomal Disorders/genetics ; Retinal Degeneration/complications ; Retinal Degeneration/genetics
    Language English
    Publishing date 2019-12-28
    Publishing country United States
    Document type Journal Article
    ISSN 2214-8019 ; 0065-2598
    ISSN (online) 2214-8019
    ISSN 0065-2598
    DOI 10.1007/978-3-030-27378-1_52
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  8. Article ; Online: Peroxisomal Disorders and Their Mouse Models Point to Essential Roles of Peroxisomes for Retinal Integrity.

    Das, Yannick / Swinkels, Daniëlle / Baes, Myriam

    International journal of molecular sciences

    2021  Volume 22, Issue 8

    Abstract: Peroxisomes are multifunctional organelles, well known for their role in cellular lipid homeostasis. Their importance is highlighted by the life-threatening diseases caused by peroxisomal dysfunction. Importantly, most patients suffering from peroxisomal ...

    Abstract Peroxisomes are multifunctional organelles, well known for their role in cellular lipid homeostasis. Their importance is highlighted by the life-threatening diseases caused by peroxisomal dysfunction. Importantly, most patients suffering from peroxisomal biogenesis disorders, even those with a milder disease course, present with a number of ocular symptoms, including retinopathy. Patients with a selective defect in either peroxisomal α- or β-oxidation or ether lipid synthesis also suffer from vision problems. In this review, we thoroughly discuss the ophthalmological pathology in peroxisomal disorder patients and, where possible, the corresponding animal models, with a special emphasis on the retina. In addition, we attempt to link the observed retinal phenotype to the underlying biochemical alterations. It appears that the retinal pathology is highly variable and the lack of histopathological descriptions in patients hampers the translation of the findings in the mouse models. Furthermore, it becomes clear that there are still large gaps in the current knowledge on the contribution of the different metabolic disturbances to the retinopathy, but branched chain fatty acid accumulation and impaired retinal PUFA homeostasis are likely important factors.
    MeSH term(s) Animals ; Disease Models, Animal ; Metabolome ; Peroxisomes/metabolism ; Phospholipids/deficiency ; Retina/metabolism ; Retina/pathology ; Retinal Diseases/metabolism ; Retinal Diseases/pathology
    Chemical Substances Phospholipids
    Language English
    Publishing date 2021-04-15
    Publishing country Switzerland
    Document type Journal Article ; Review
    ZDB-ID 2019364-6
    ISSN 1422-0067 ; 1422-0067 ; 1661-6596
    ISSN (online) 1422-0067
    ISSN 1422-0067 ; 1661-6596
    DOI 10.3390/ijms22084101
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  9. Article ; Online: Peroxisomes Are Critical for the Development and Maintenance of B1 and Marginal Zone B Cells but Dispensable for Follicular B Cells and T Cells.

    Muri, Jonathan / Corak, Basak / Matsushita, Mai / Baes, Myriam / Kopf, Manfred

    Journal of immunology (Baltimore, Md. : 1950)

    2022  Volume 208, Issue 4, Page(s) 839–850

    Abstract: Antioxidant systems maintain cellular redox (oxidation-reduction) homeostasis. In contrast with other key redox pathways, such as the thioredoxin system, glutathione, and NF-E2-related factor 2 (Nrf2), little is known about the function of the redox- ... ...

    Abstract Antioxidant systems maintain cellular redox (oxidation-reduction) homeostasis. In contrast with other key redox pathways, such as the thioredoxin system, glutathione, and NF-E2-related factor 2 (Nrf2), little is known about the function of the redox-sensitive organelle "peroxisome" in immune cells. In this study, we show that the absence of peroxisomes in conditional
    MeSH term(s) Animals ; Antibody Formation/immunology ; B-Lymphocyte Subsets/immunology ; B-Lymphocyte Subsets/metabolism ; Biomarkers ; Cell Differentiation ; Disease Susceptibility ; Germinal Center/immunology ; Germinal Center/metabolism ; Host-Pathogen Interactions/genetics ; Host-Pathogen Interactions/immunology ; Immunization ; Immunophenotyping ; Lymphoid Tissue/immunology ; Lymphoid Tissue/metabolism ; Lymphopoiesis/genetics ; Mice ; Mice, Knockout ; Oxidation-Reduction ; Oxidative Stress ; Peroxisome-Targeting Signal 1 Receptor/deficiency ; Peroxisomes/metabolism ; Pneumococcal Infections/immunology ; Pneumococcal Infections/microbiology ; Streptococcus pneumoniae/immunology ; T-Lymphocyte Subsets/immunology ; T-Lymphocyte Subsets/metabolism
    Chemical Substances Biomarkers ; Peroxisome-Targeting Signal 1 Receptor ; Pex5 protein, mouse
    Language English
    Publishing date 2022-01-24
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 3056-9
    ISSN 1550-6606 ; 0022-1767 ; 1048-3233 ; 1047-7381
    ISSN (online) 1550-6606
    ISSN 0022-1767 ; 1048-3233 ; 1047-7381
    DOI 10.4049/jimmunol.2100518
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Ud II Zs.37: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular
    ab Jg. 2022: Lesesaal (EG)
    Zs.MG 28: Show issues

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  10. Article ; Online: Enhanced Levels of Peroxisome-Derived H2O2 Do Not Induce Pexophagy but Impair Autophagic Flux in HEK-293 and HeLa Cells

    Li, Hongli / Lismont, Celien / Costa, Cláudio F. / Hussein, Mohamed A. F. / Baes, Myriam / Fransen, Marc

    Antioxidants. 2023 Mar. 02, v. 12, no. 3

    2023  

    Abstract: 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 ... ...

    Abstract 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 processes, including autophagy. However, it remains unclear whether changes in peroxisomal H₂O₂ (po-H₂O₂) emission impact the autophagic process and to which extent peroxisomes with a disturbed H₂O₂ metabolism are selectively eliminated through a process called “pexophagy”. To address these issues, we generated and validated HEK-293 and HeLa pexophagy reporter cell lines in which the production of po-H₂O₂ can be modulated. We demonstrate that (i) po-H₂O₂ can oxidatively modify multiple selective autophagy receptors and core autophagy proteins, (ii) neither modest nor robust levels of po-H₂O₂ emission act as a prime determinant of pexophagy, and (iii) high levels of po-H₂O₂ impair autophagic flux by oxidative inhibition of enzymes involved in LC3II formation. Unexpectedly, our analyses also revealed that the autophagy receptor optineurin can be recruited to peroxisomes, thereby triggering pexophagy. In summary, these findings lend support to the idea that, during cellular and organismal aging, peroxisomes with enhanced H₂O₂ release can escape pexophagy and downregulate autophagic activity, thereby perpetuating the accumulation of damaged and toxic cellular debris.
    Keywords hydrogen peroxide ; macroautophagy ; metabolism ; oxidants ; peroxisomes ; toxicity
    Language English
    Dates of publication 2023-0302
    Publishing place Multidisciplinary Digital Publishing Institute
    Document type Article ; Online
    ZDB-ID 2704216-9
    ISSN 2076-3921
    ISSN 2076-3921
    DOI 10.3390/antiox12030613
    Database NAL-Catalogue (AGRICOLA)

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