We found a match
Your institution may have access to this item. Find your institution then sign in to continue.
- Title
Characterisation of Fmrp in zebrafish: evolutionary dynamics of thefmr1gene.
- Authors
Padje, Sandra; Engels, Bart; Blonden, Lau; Severijnen, Lies-Anne; Verheijen, Frans; Oostra, Ben; Willemsen, Rob
- Abstract
Fragile X syndrome is the most common inherited form of mental retardation. It is caused by the lack of the Fragile X Mental Retardation Protein (FMRP), which is encoded by theFMR1gene. AlthoughFmr1knockout mice display some characteristics also found in fragile X patients, it is a complex animal model to study brain abnormalities, especially during early embryonic development. Interestingly, the ortholog of theFMR1gene has been identified not only in mouse, but also in zebrafish (Danio rerio). In this study, an amino acid sequence comparison of FMRP orthologs was performed to determine the similar regions of FMRP between several species, including human, mouse, frog, fruitfly and zebrafish. Further characterisation of Fmrp has been performed in both adults and embryos of zebrafish using immunohistochemistry and western blotting with specific antibodies raised against zebrafish Fmrp. We have demonstrated a strong Fmrp expression in neurons of the brain and only a very weak expression in the testis. In brain tissue, a different distribution of the isoforms of Fmrp, compared to human and mouse brain tissue, was shown using western blot analysis. Due to the high similarity between zebrafish Fmrp and human FMRP and their similar expression pattern, the zebrafish has great potential as a complementary animal model to study the pathogenesis of the fragile X syndrome, especially during embryonic development.
- Subjects
FRAGILE X syndrome; INTELLECTUAL disabilities; ZEBRA danio; FISHES; GENES
- Publication
Development Genes & Evolution, 2005, Vol 215, Issue 4, p198
- ISSN
0949-944X
- Publication type
Article
- DOI
10.1007/s00427-005-0466-0