Nature Medicine
3, 646 - 650 (1997)
doi:10.1038/nm0697-646
Enx (Hox11L1)-deficient mice develop myenteric neuronal hyperplasia and megacolonSenji Shirasawa1, Anne Marie R. Yunker2, Kevin A. Roth2, Gary A. Brown1, Susan Horning1
& Stanley J. Korsmeyer1, 3
1Howard Hughes Medical Institute, Division of Molecular Oncology, Department of Medicine, Washington University School of Medicine, 660 South Euclid, Box 8022, St. Louis, Missouri 63110, USA
2Department of Pathology, Washington University School of Medicine, 660 South Euclid, Box 8118, St. Louis, Missouri 63110,USA
3Correspondence should be addressed to S.J.K. 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 differentiate cell fate of enteric neurons.
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