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- Title
A novel mouse model of intestinal neuronal dysplasia: visualization of the enteric nervous system.
- Authors
Fujiwara, Naho; Miyahara, Katsumi; Lee, Dorothy; Nakazawa-Tanaka, Nana; Akazawa, Chihiro; Hatano, Masahiko; Pierro, Agostino; Yamataka, Atsuyuki
- 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.
- Publication
Pediatric Surgery International, 2023, Vol 39, Issue 1, p1
- ISSN
0179-0358
- Publication type
Article
- DOI
10.1007/s00383-023-05585-w