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Research Article

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

M Hatano, T Aoki, M Dezawa, S Yusa, Y Iitsuka, H Koseki, M Taniguchi and T Tokuhisa

Division of Developmental Genetics, Chiba University School of Medicine, Chiba 260, Japan. hatano@med.m.chiba-u.ac.jp

Published August 15, 1997

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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