title: Phox2b function in the enteric nervous system is conserved in zebrafish and is sox10-dependent.
abstract: Zebrafish lacking functional sox10 have defects in non-ectomesenchymal neural crest derivatives including the enteric nervous system (ENS) and as such provide an animal model for human Waardenburg Syndrome IV. Here, we characterize zebrafish phox2b as a functionally conserved marker of the developing ENS. We show that morpholino-mediated knockdown of Phox2b generates fish modeling Hirschsprung disease. Using markers, including phox2b, we investigate the ontogeny of the sox10 ENS phenotype. As previously shown for melanophore development, ENS progenitor fate specification fails in these mutant fish. However, in addition, we trace back the sox10 mutant ENS defect to an even earlier time point, finding that most neural crest cells fail to migrate ventrally to the gut primordium.
authors.affiliation: Centre for Regenerative Medicine, Developmental Biology Programme, Department of Biology and Biochemistry, University of Bath, Claverton Down, Bath BA2 7AY, UK.
authors.forename: Stone
authors.initials: S
authors.lastname: Elworthy
authors.forename: Jorge P
authors.initials: JP
authors.lastname: Pinto
authors.forename: Anna
authors.initials: A
authors.lastname: Pettifer
authors.forename: M Leonor
authors.initials: ML
authors.lastname: Cancela
authors.forename: Robert N
authors.initials: RN
authors.lastname: Kelsh
mesh_terms: D000595:Amino Acid Sequence; D000818:Animals; D002352:Carrier Proteins / Q000235:genetics* / Q000378:metabolism / Q000502:physiology*; D002490:Central Nervous System / Q000196:embryology; D002874:Chromosome Mapping; D017615:Enteric Nervous System / Q000196:embryology*; D018507:Gene Expression Regulation, Developmental; D006609:High Mobility Group Proteins / Q000235:genetics* / Q000378:metabolism / Q000502:physiology*; D018398:Homeodomain Proteins / Q000378:metabolism / Q000502:physiology*; D017403:In Situ Hybridization; D008547:Melanophores / Q000378:metabolism; D008856:Microscopy, Fluorescence; D008957:Models, Genetic; D008969:Molecular Sequence Data; D009154:Mutation; D009432:Neural Crest / Q000378:metabolism; D009474:Neurons / Q000378:metabolism; D010641:Phenotype; D010802:Phylogeny; D055757:SOXE Transcription Factors; D017386:Sequence Homology, Amino Acid; D013234:Stem Cells / Q000378:metabolism; D013997:Time Factors; D014157:Transcription Factors / Q000378:metabolism / Q000502:physiology*; D015027:Zebrafish; D029961:Zebrafish Proteins / Q000235:genetics* / Q000378:metabolism / Q000502:physiology*
chemical_list: D002352:Carrier Proteins; D006609:High Mobility Group Proteins; D018398:Homeodomain Proteins; C109220:NBPhox protein; D055757:SOXE Transcription Factors; D014157:Transcription Factors; D029961:Zebrafish Proteins; C442229:sox10 protein, zebrafish