Enx (Hox11L1)-deficient mice develop myenteric neuronal hyperplasia and megacolon

被引:105
作者
Shirasawa, S
Yunker, AMR
Roth, KA
Brown, GA
Horning, S
Korsmeyer, SJ
机构
[1] WASHINGTON UNIV,SCH MED,DEPT MED,HOWARD HUGHES MED INST,DIV MOL ONCOL,ST LOUIS,MO 63110
[2] WASHINGTON UNIV,SCH MED,DEPT PATHOL,ST LOUIS,MO 63110
关键词
D O I
10.1038/nm0697-646
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
The isolated homeobox gene Enx (Hox11L1) is expressed in enteric neurons Innervating distal ileum, and proximal and distal colon. Enx-deficient mice develop megacolon with massive distension of the proximal colon. The number of myenteric ganglia, total neurons per ganglion, and NADPH diaphorase presumptive inhibitory neurons per ganglion are increased in the proximal and distal colon, but decreased in the distal ileum of all Enx(-/-) mice. Enx(-/-) mice provide a model for human neuronal intestinal dysplasia (NID), in which myenteric neuronal hyperplasia and megacolon are seen. These results suggest that Enx is required for the proper positional specification and differentiative cell fate of enteric neurons.
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页码:646 / 650
页数:5
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