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

Masahiko Hatano, Taito Aoki, Mari Dezawa, Seiichi Yusa, Yoshinori Iitsuka, Haruhiko Koseki, Masaru Taniguchi, Takeshi Tokuhisa

Research output: Contribution to journalArticle

91 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)795-801
Number of pages7
JournalJournal of Clinical Investigation
Volume100
Issue number4
DOIs
Publication statusPublished - 1997 Aug 15

Keywords

  • Enteric ganglia
  • Gene targeting
  • Homeobox
  • Hyperinnervation
  • NADPH diaphorase

ASJC Scopus subject areas

  • Medicine(all)

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    Hatano, M., Aoki, T., Dezawa, M., Yusa, S., Iitsuka, Y., Koseki, H., Taniguchi, M., & Tokuhisa, T. (1997). A novel pathogenesis of megacolon in Ncx/Hox11L.1 deficient mice. Journal of Clinical Investigation, 100(4), 795-801. https://doi.org/10.1172/JCI119593