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

被引:81
作者
Hatano, M
Aoki, T
Dezawa, M
Yusa, S
Iitsuka, Y
Koseki, H
Taniguchi, M
Tokuhisa, T
机构
[1] CHIBA UNIV,CTR BIOMED SCI,DIV MOL IMMUNOL,CHUO KU,CHIBA 260,JAPAN
[2] CHIBA UNIV,SCH MED,DEPT ANAT,CHUO KU,CHIBA 260,JAPAN
关键词
gene targeting; homeobox; enteric ganglia; NADPH diaphorase; hyperinnervation;
D O I
10.1172/JCI119593
中图分类号
R-3 [医学研究方法]; R3 [基础医学];
学科分类号
1001 ;
摘要
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.
引用
收藏
页码:795 / 801
页数:7
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