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Publication : A novel pathogenesis of megacolon in Ncx/Hox11L.1 deficient mice.

First Author  Hatano M Year  1997
Journal  J Clin Invest Volume  100
Issue  4 Pages  795-801
PubMed ID  9259577 Mgi Jnum  J:42356
Mgi Id  MGI:1095635 Doi  10.1172/JCI119593
Citation  Hatano M, et al. (1997) A novel pathogenesis of megacolon in Ncx/Hox11L.1 deficient mice. J Clin Invest 100(4):795-801
abstractText  The Ncx/Hox11L.1 gene, a member of the Hox11 homeobox gene family, is mainly expressed in neural crest-derived tissues. To elucidate the role of Ncx/Hox11L.1, the gene has been inactivated in embryonic stem cells by homologous recombination. The homozygous mutant mice were viable. These mice developed megacolon with enteric ganglia by age 3-5 wk. Histochemical analysis of the ganglia revealed that the enteric neurons hyperinnervated in the narrow segment of megacolon. Some of these neuronal cells degenerated and neuronal cell death occurred in later stages. We propose that Ncx/Hox11L.1 is required for maintenance of proper functions of the enteric nervous system. These mutant mice can be used to elucidate a novel pathogenesis for human neuronal intestinal dysplasia.
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