Modelling Peroxisomal Disorders in Zebrafish
Peroxisomes are ubiquitous, dynamic, oxidative organelles with key functions in cellular lipid metabolism and redox homeostasis. They have been linked to healthy ageing, neurodegeneration, cancer, the combat of pathogens and viruses, and infection and immune responses. Their biogenesis relies on sev...
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MDPI AG
2025-01-01
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| author | Chenxing S. Jiang Michael Schrader |
| author_facet | Chenxing S. Jiang Michael Schrader |
| author_sort | Chenxing S. Jiang |
| collection | DOAJ |
| description | Peroxisomes are ubiquitous, dynamic, oxidative organelles with key functions in cellular lipid metabolism and redox homeostasis. They have been linked to healthy ageing, neurodegeneration, cancer, the combat of pathogens and viruses, and infection and immune responses. Their biogenesis relies on several peroxins (encoded by <i>PEX</i> genes), which mediate matrix protein import, membrane assembly, and peroxisome multiplication. Defects in peroxins or peroxisomal enzymes can result in severe disorders, including developmental and neurological abnormalities. The drive to understand the role of peroxisomes in human health and disease, as well as their functions in tissues and organs or during development, has led to the establishment of vertebrate models. The zebrafish (<i>Danio rerio</i>) has become an attractive vertebrate model organism to investigate peroxisomal functions. Here, we provide an overview of the visualisation of peroxisomes in zebrafish, as well as the peroxisomal metabolic functions and peroxisomal protein inventory in comparison to human peroxisomes. We then present zebrafish models which have been established to investigate peroxisomal disorders. These include model zebrafish for peroxisome biogenesis disorders/Zellweger Spectrum disorders, and single enzyme deficiencies, particularly adrenoleukodystrophy and fatty acid beta-oxidation abnormalities. Finally, we highlight zebrafish models for deficiencies of dually targeted peroxisomal/mitochondrial proteins. Advantages for the investigation of peroxisomes during development and approaches to the application of zebrafish models for drug screening are discussed. |
| format | Article |
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| institution | Kabale University |
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| language | English |
| publishDate | 2025-01-01 |
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| series | Cells |
| spelling | doaj-art-9827d5d11a074c30ba6bf7dbc68ff7272025-01-24T13:26:51ZengMDPI AGCells2073-44092025-01-0114214710.3390/cells14020147Modelling Peroxisomal Disorders in ZebrafishChenxing S. Jiang0Michael Schrader1Biosciences, Faculty of Health and Life Sciences, University of Exeter, Exeter EX4 4QD, UKBiosciences, Faculty of Health and Life Sciences, University of Exeter, Exeter EX4 4QD, UKPeroxisomes are ubiquitous, dynamic, oxidative organelles with key functions in cellular lipid metabolism and redox homeostasis. They have been linked to healthy ageing, neurodegeneration, cancer, the combat of pathogens and viruses, and infection and immune responses. Their biogenesis relies on several peroxins (encoded by <i>PEX</i> genes), which mediate matrix protein import, membrane assembly, and peroxisome multiplication. Defects in peroxins or peroxisomal enzymes can result in severe disorders, including developmental and neurological abnormalities. The drive to understand the role of peroxisomes in human health and disease, as well as their functions in tissues and organs or during development, has led to the establishment of vertebrate models. The zebrafish (<i>Danio rerio</i>) has become an attractive vertebrate model organism to investigate peroxisomal functions. Here, we provide an overview of the visualisation of peroxisomes in zebrafish, as well as the peroxisomal metabolic functions and peroxisomal protein inventory in comparison to human peroxisomes. We then present zebrafish models which have been established to investigate peroxisomal disorders. These include model zebrafish for peroxisome biogenesis disorders/Zellweger Spectrum disorders, and single enzyme deficiencies, particularly adrenoleukodystrophy and fatty acid beta-oxidation abnormalities. Finally, we highlight zebrafish models for deficiencies of dually targeted peroxisomal/mitochondrial proteins. Advantages for the investigation of peroxisomes during development and approaches to the application of zebrafish models for drug screening are discussed.https://www.mdpi.com/2073-4409/14/2/147peroxisomeslipid metabolismPEXorganelle biogenesismetabolic disordersfatty acid beta-oxidation |
| spellingShingle | Chenxing S. Jiang Michael Schrader Modelling Peroxisomal Disorders in Zebrafish Cells peroxisomes lipid metabolism PEX organelle biogenesis metabolic disorders fatty acid beta-oxidation |
| title | Modelling Peroxisomal Disorders in Zebrafish |
| title_full | Modelling Peroxisomal Disorders in Zebrafish |
| title_fullStr | Modelling Peroxisomal Disorders in Zebrafish |
| title_full_unstemmed | Modelling Peroxisomal Disorders in Zebrafish |
| title_short | Modelling Peroxisomal Disorders in Zebrafish |
| title_sort | modelling peroxisomal disorders in zebrafish |
| topic | peroxisomes lipid metabolism PEX organelle biogenesis metabolic disorders fatty acid beta-oxidation |
| url | https://www.mdpi.com/2073-4409/14/2/147 |
| work_keys_str_mv | AT chenxingsjiang modellingperoxisomaldisordersinzebrafish AT michaelschrader modellingperoxisomaldisordersinzebrafish |