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Phox2b function in the enteric nervous system is conserved in zebrafish and is sox10-dependent
Stone Elworthy1, Jorge P Pinto, Anna Pettifer
1Centre for Regenerative Medicine, Developmental Biology Programme, Department of Biology and Biochemistry, University of Bath, Claverton Down, Bath BA2 7AY, UK.
Mechanisms of Development
|April 9, 2005
Summary
Zebrafish sox10 mutants exhibit enteric nervous system (ENS) defects, modeling Waardenburg Syndrome IV. Phox2b knockdown also impairs ENS development, revealing neural crest migration failure in sox10 mutants.
Area of Science:
- Developmental biology
- Genetics
- Neuroscience
Background:
- Sox10 is crucial for neural crest development, and its absence causes defects in non-ectomesenchymal derivatives like the enteric nervous system (ENS).
- Mutations in SOX10 are linked to Waardenburg Syndrome IV in humans, a disorder affecting pigmentation and the ENS.
Purpose of the Study:
- To characterize phox2b as a marker for the developing zebrafish ENS.
- To investigate the developmental origins of ENS defects in sox10 mutant zebrafish.
- To model Hirschsprung disease using phox2b knockdown in zebrafish.
Main Methods:
- Morpholino-mediated knockdown of phox2b in zebrafish embryos.
- Analysis of sox10 mutant zebrafish using specific molecular markers.
- Tracing neural crest cell migration patterns during embryonic development.
Main Results:
- Phox2b knockdown in zebrafish recapitulates key features of Hirschsprung disease.
- sox10 mutant zebrafish display defects in ENS progenitor fate specification.
- sox10 mutant zebrafish show a significant failure of neural crest cells to migrate to the gut primordium.
Conclusions:
- Phox2b is a conserved marker for the developing zebrafish ENS.
- The sox10 ENS phenotype originates from a failure in early neural crest migration, preceding fate specification defects.
- Zebrafish sox10 mutants serve as a valuable model for studying ENS development and Waardenburg Syndrome IV.