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Interactions between enteric neural crest-derived cells (ENCC) and the surrounding intestinal microenvironment, such as the extracellular matrix (ECM), are critical for regulating enteric nervous system (ENS) development. Integrins are the major receptors for ECM molecules, such as laminin, which have been reported to be involved in the pathogenesis of Hirschsprung's disease. In this study, we examined the expression of β1 integrin in the endothelin receptor B (Ednrb) knock out (KO) mouse gut, which presents with an aganglionic colon. A Sox10-Venus-positive Ednrb KO mouse, where ENCC is labeled with fluorescent protein, 'Venus', was created. Sox10-Venus-positive Ednrb wild type (WT) were used as controls. Small intestine, proximal colon and distal colon were dissected on E13.5 and E15.5 and β1 integrin expression of the gut tissue was examined by immunohistochemistry and real time RT-PCR. The cells of the gut dissected on E11.5 were isolated and cultured for 2 days. Venus-positive ENCC were immunostained with β1 integrin and Tuj-1, which is a marker for neurons. The expression of β1 integrin was not significantly different between KO and WT in all parts of the gut examined. However, the β1 integrin expression in the isolated ENCC was significantly decreased in KO compared to WT. The average threshold area was 42.98 ± 17.47% in KO and 73.53 ± 13.77 in WT (p < 0.001). We demonstrated that β1 integrin expression was specifically decreased in ENCC in Ednrb KO mice. Our results suggest that impaired interaction between integrin and its ligands may disturb normal ENS development, resulting in an aganglionic colon.


Nana Nakazawa-Tanaka, Katsumi Miyahara, Naho Fujiwara, Takanori Ochi, Ryo Sueyoshi, Shuko Nojiri, Chihiro Akazawa, Masahiko Urao, Atsuyuki Yamataka. Decreased expression of β1 integrin in enteric neural crest cells of the endothelin receptor B null mouse model. Pediatric surgery international. 2020 Jan;36(1):43-48

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PMID: 31576467

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