Molecules and Cells

Korean Society for Molecular and Cellular Biology (한국분자세포생물학회)
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Developmental Roles of D-bifunctional Protein-A Zebrafish Model of Peroxisome Dysfunction

  • Kim, Yong-Il (Center for Metabolic Function Regulation, and Department of Microbiology, School of Medicine, Wonkwang University) ;
  • Bhandari, Sushil (Center for Metabolic Function Regulation, and Department of Microbiology, School of Medicine, Wonkwang University) ;
  • Lee, Joon No (Center for Metabolic Function Regulation, and Department of Microbiology, School of Medicine, Wonkwang University) ;
  • Yoo, Kyeong-Won (Center for Metabolic Function Regulation, and Department of Microbiology, School of Medicine, Wonkwang University) ;
  • Kim, Se-Jin (Center for Metabolic Function Regulation, and Department of Microbiology, School of Medicine, Wonkwang University) ;
  • Oh, Gi-Su (Center for Metabolic Function Regulation, and Department of Microbiology, School of Medicine, Wonkwang University) ;
  • Kim, Hyung-Jin (Center for Metabolic Function Regulation, and Department of Microbiology, School of Medicine, Wonkwang University) ;
  • Cho, Meyoung (Department of Internal Medicine, Gunsan Medical Center) ;
  • Kwak, Jong-Young (Immune-network Pioneer Research Center, Department of Biochemistry, College of Medicine, Dong-A University) ;
  • So, Hong-Seob (Center for Metabolic Function Regulation, and Department of Microbiology, School of Medicine, Wonkwang University) ;
  • Park, Raekil (Center for Metabolic Function Regulation, and Department of Microbiology, School of Medicine, Wonkwang University) ;
  • Choe, Seong-Kyu (Center for Metabolic Function Regulation, and Department of Microbiology, School of Medicine, Wonkwang University)
  • Received : 2013.10.17
  • Accepted : 2013.11.08
  • Published : 2014.01.31
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Abstract

The peroxisome is an intracellular organelle that responds dynamically to environmental changes. Various model organisms have been used to study the roles of peroxisomal proteins in maintaining cellular homeostasis. By taking advantage of the zebrafish model whose early stage of embryogenesis is dependent on yolk components, we examined the developmental roles of the D-bifunctional protein (Dbp), an essential enzyme in the peroxisomal ${\beta}$-oxidation. The knockdown of dbp in zebrafish phenocopied clinical manifestations of its deficiency in human, including defective craniofacial morphogenesis, growth retardation, and abnormal neuronal development. Overexpression of murine Dbp rescued the morphological phenotypes induced by dbp knockdown, indicative of conserved roles of Dbp during zebrafish and mammalian development. Knockdown of dbp impaired normal development of blood, blood vessels, and most strikingly, endoderm-derived organs including the liver and pancreas - a phenotype not reported elsewhere in connection with peroxisome dysfunction. Taken together, our results demonstrate for the first time that zebrafish might be a useful model animal to study the role of peroxisomes during vertebrate development.

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References

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