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The role of SOX10 during enteric nervous system development
Nadege Bondurand1, Mai Har Sham
1INSERM, U955, Equipe 11, Hôpital Henri Mondor, 51 Avenue du Maréchal de Lattre de Tassigny, F-94000 Créteil, France; Université Paris Est, UMR_S955, UPEC, Créteil, F-94000 Créteil, France.
Developmental Biology
|May 7, 2013
Summary
SOX10 is crucial for neural crest cell development and function, impacting the enteric nervous system and causing diseases like Waardenburg-Hirschsprung syndrome. Understanding its interactions is key to future research and treatments.
Area of Science:
- Developmental Biology
- Genetics
- Neuroscience
Background:
- SOX10 is a key transcription factor for neural crest (NC) progenitors and their derivatives.
- Its role is implicated in Waardenburg-Hirschsprung disease, affecting pigmentation, hearing, and the enteric nervous system (ENS).
Purpose of the Study:
- To review the expression, regulation, and function of SOX10 in ENS development and disease.
- To explore SOX10's genetic and molecular interactions with other ENS factors.
- To identify future research directions for SOX10 in enteric neural crest cells (ENCCs).
Main Methods:
- Literature review of SOX10's role in NC cells, melanocytes, and ENS.
- Analysis of studies on SOX10 mutations, animal models, and cell-based experiments.
- Examination of genetic modifiers and molecular interactions of SOX10.
Main Results:
- SOX10 is vital for maintaining NC progenitor numbers and determining glial cell fate.
- Phenotypic variability in SOX10-related diseases is linked to cooperative interactions with other factors.
- Numerous genetic modifiers of SOX10 have been identified.
Conclusions:
- SOX10 is essential for normal ENS development and its dysfunction leads to disease.
- Understanding SOX10's interactions with partner factors and the niche environment is critical.
- Future genomic and cell biological studies will advance knowledge of SOX10 function.
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