A novel mouse model of intestinal neuronal dysplasia: visualization of the enteric nervous system
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Read the article on link.springer.com ↗Article · Nov 2023 · 1 min read
In brief
In brief
This study establishes a novel Sox10-Venus+/Ncx−/− transgenic mouse model that replicates intestinal neuronal dysplasia phenotypes, including megacolon and abnormal enteric ganglia. The model enables real-time visualization of the enteric nervous system without staining, providing a cost-effective tool for studying IND pathogenesis.
Written by the GCMD Library team from the article.
Abstract
Purpose
Intestinal neuronal dysplasia (IND) is a congenital anomaly affecting gastrointestinal neural innervation, but the pathogenesis remains unclear. The homozygous Ncx/Hox11L.1 knockout (Ncx−/−) mice exhibit megacolon and enteric ganglia anomalies, resembling IND phenotypes. Sox10-Venus transgenic mouse were used to visualize enteric neural crest cells in real time. This study aims to establish a novel mouse model of Sox10-Venus+/Ncx−/− mouse to study the pathogenesis of IND.
Methods
Sox10-Venus+/Ncx−/− (Ncx−/−) (n = 8) mice and Sox10-Venus+/Ncx+/+ controls (control) (n = 8) were euthanized at 4–5 weeks old, and excised intestines were examined with fluorescence microscopy. Immunohistochemistry was performed on tissue sections with neural marker Tuj1.
Results
Ncx−/− mice exhibited dilated cecum and small intestine. Body weight of Ncx−/− mice was lower with higher ratio of small intestine length relative to body weight. The neural network (Sox10-Venus) was observed along the intestine wall in Ncx−/− and control mice without staining. Ectopic and increased expression of Tuj1 was observed in both small intestine and proximal colon of Ncx−/− mice.
Conclusion
This study has established a reliable animal model that exhibits characteristics similar to patients with IND. This novel mouse model can allow the easy visualization of ENS in a time- and cost-effective way to study the pathogenesis of IND.