Developmental roles of D-bifunctional protein-A zebrafish model of peroxisome dysfunction

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 dep...

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Published in:Molecules and cells 2014, 37(1), , pp.74-80
Main Authors: Kim, Yong-Il, Bhandari, Sushil, Lee, Joon No, Yoo, Kyeong-Won, Kim, Se-Jin, Oh, Gi-Su, Kim, Hyung-Jin, Cho, Meyoung, Kwak, Jong-Young, So, Hong-Seob, Park, Raekil, Choe, Seong-Kyu
Format: Article
Language:English
Subjects:
Amino Acid Sequence
Animals
Conserved Sequence
Disease Models, Animal
Embryonic Development
Gastrointestinal Tract - abnormalities
Gene Expression
Gene Knockdown Techniques
Genetic Complementation Test
Humans
Mice
Molecular Sequence Data
Neurogenesis
Peroxisomal Disorders - enzymology
Peroxisomal Disorders - genetics
Peroxisomal Multifunctional Protein-2 - genetics
Peroxisomal Multifunctional Protein-2 - metabolism
Peroxisomes - enzymology
Yolk Sac - metabolism
Zebrafish - embryology
Zebrafish - genetics
Zebrafish Proteins - genetics
Zebrafish Proteins - metabolism
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Summary: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 β-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.
ISSN:1016-8478
0219-1032
DOI:10.14348/molcells.2014.2300