PUBLICATION

Aberrant expression of genes necessary for neuronal development and notch signaling in an epileptic mind bomb zebrafish

Authors
Hortopan, G.A., and Baraban, S.C.
ID
ZDB-PUB-110629-14
Date
2011
Source
Developmental Dynamics : an official publication of the American Association of Anatomists   240(8): 1964-76 (Journal)
Registered Authors
Baraban, Scott
Keywords
brain, development, GFP, notch, zebrafish
MeSH Terms
  • Animals
  • Animals, Genetically Modified
  • Embryo, Nonmammalian/anatomy & histology
  • Embryo, Nonmammalian/physiology
  • Epilepsy/physiopathology*
  • Gene Expression Profiling
  • Gene Expression Regulation, Developmental*
  • Homeodomain Proteins/genetics
  • Homeodomain Proteins/metabolism
  • Microarray Analysis
  • Neurogenesis/physiology
  • Neurons/cytology
  • Neurons/physiology*
  • Receptors, Notch/genetics
  • Receptors, Notch/metabolism*
  • Signal Transduction/genetics*
  • Ubiquitin-Protein Ligases/genetics
  • Ubiquitin-Protein Ligases/metabolism*
  • Zebrafish/anatomy & histology
  • Zebrafish/embryology
  • Zebrafish/physiology*
  • Zebrafish Proteins/genetics
  • Zebrafish Proteins/metabolism*
(all 23)
PubMed
21688347 Full text @ Dev. Dyn.
Abstract
Mutation within an ubiquitin E3 ligase gene can lead to a failure in Notch signaling, excessive neurons, and depletion of neural progenitor cells in mind bomb mutants. Using mib(hi904) zebrafish, we reported seizures and a down-regulation of γ-aminobutyric acid (GABA) signaling pathway genes. A transcriptome analysis also identified differential expression pattern of genes related to Notch signaling and neurodevelopment. Here, we selected nine of these genes (her4.2, hes5, bhlhb5, hoxa5a, hoxb5b, dmbx1a, dbx1a, nxph1, and plxnd1) and performed a more thorough analysis of expression using conventional polymerase chain reaction, real-time polymerase chain reaction and in situ hybridization. Transgenic reporter fish (Gfap:GFP and Dlx5a-6a:GFP) were used to assess early brain morphology in vivo. Down-regulation of many of these genes was prominent throughout key structures of the developing mib(hi904) zebrafish brain including, but not limited to, the pallium, ventral thalamus, and optic tectum. Brain expression of Dlx5a-6a and Gfap was also reduced. In conclusion, these expression studies indicate a general down-regulation of Notch signaling genes necessary for proper brain development and suggest that these mutant fish could provide valuable insights into neurological conditions, such as Angelman syndrome, associated with ubiquitin E3 ligase mutation.
Genes / Markers
Marker Marker Type Name
actb1GENEactin, beta 1
bhlhe22GENEbasic helix-loop-helix family, member e22
dbx1aGENEdeveloping brain homeobox 1a
dmbx1aGENEdiencephalon/mesencephalon homeobox 1a
gfapGENEglial fibrillary acidic protein
her15.1GENEhairy and enhancer of split-related 15, tandem duplicate 1
her4.2GENEhairy-related 4, tandem duplicate 2
hoxa5aGENEhomeobox A5a
hoxb5bGENEhomeobox B5b
mib1GENEMIB E3 ubiquitin protein ligase 1
1 - 10 of 12
Show
Figures
Figure Gallery (10 images)
Show all Figures
Expression
Phenotype
Fish Conditions Stage Phenotype Figure
mib1hi904Tg/hi904Tg (AB)standard conditionsProtruding-mouth
mib1hi904Tg/hi904Tg (AB)standard conditionsProtruding-mouth
1 - 2 of 2
Show
Mutations / Transgenics
Allele Construct Type Affected Genomic Region
hi904TgTransgenic Insertion
mi2001TgTransgenic Insertion
    ot1TgTransgenic Insertion
      1 - 3 of 3
      Show
      Human Disease / Model
      No data available
      Sequence Targeting Reagents
      No data available
      Fish
      Antibodies
      No data available
      Orthology
      Engineered Foreign Genes
      Marker Marker Type Name
      GFPEFGGFP
      1 - 1 of 1
      Show
      Mapping
      Your Input Welcome
      We welcome your input and comments. Please use this form to recommend updates to the information in ZFIN. We appreciate as much detail as possible and references as appropriate. We will review your comments promptly.
      Citation
      Hortopan, G.A., and Baraban, S.C. (2011) Aberrant expression of genes necessary for neuronal development and notch signaling in an epileptic mind bomb zebrafish. Developmental Dynamics : an official publication of the American Association of Anatomists. 240(8):1964-76.