Enx (Hox11L1)-deficient mice develop myenteric neuronal hyperplasia and megacolon.
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.
Shirasawa
S
S
Department of Medicine, Washington University School of Medicine, St. Louis, Missouri 63110, USA.
Yunker
A M
AM
Roth
K A
KA
Brown
G A
GA
Horning
S
S
Korsmeyer
S J
SJ
D000818:Animals; D003106:Colon / Q000378:metabolism / Q000473:pathology*; D017615:Enteric Nervous System / Q000473:pathology*; D005801:Genes, Homeobox / Q000502:physiology*; D018398:Homeodomain Proteins / Q000378:metabolism / Q000502:physiology*; D006965:Hyperplasia; D007082:Ileum / Q000378:metabolism / Q000473:pathology*; D017403:In Situ Hybridization; D008531:Megacolon / Q000235:genetics*; D051379:Mice; D008810:Mice, Inbred C57BL; D018345:Mice, Knockout; D009252:NADPH Dehydrogenase / Q000378:metabolism; D015513:Oncogene Proteins / Q000378:metabolism / Q000502:physiology*; D012737:Sex Factors; D013997:Time Factors
D018398:Homeodomain Proteins; D015513:Oncogene Proteins; C487378:Tlx1 protein, mouse; D009252:NADPH Dehydrogenase