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Intestinal neuronal dysplasia-like pathology in Ncx/Hox11L.1 gene-deficient mice.
- Source :
-
Journal of pediatric surgery [J Pediatr Surg] 2001 Aug; Vol. 36 (8), pp. 1293-6. - Publication Year :
- 2001
-
Abstract
- Background/purpose: Ncx/Hox11L.1-deficient (Ncx-/-) mice specifically created by the authors had mega-ileo-ceco-colon (mega-ICC) with a caliber change in the proximal colon. The authors studied the nerve distribution in the bowel of these Ncx-/- mice to determine the cause of their bowel dysmotility.<br />Methods: Four-week-old Ncx-/- mice (n = 10; 5 with mega-ICC, 5 without mega-ICC) were killed and the bowel harvested. Half of each specimen was snap frozen for AchE and NADPH-diaphorase histochemistry, and the other half were fixed with 10% formalin for H&E staining and immunohistochemistry using PGP9.5 antibody (a marker for neurons), C-kit antibody (a marker for intestinal pacemaker cells), and stem cell factor antibody (a marker for C-kit ligand). Age-matched wild-type normal mice (n = 5) served as controls.<br />Results: In the ileum, cecum, and proximal colon from all Ncx-/- mice (irrespective of the association of mega-ICC), typical findings of human intestinal neuronal dysplasia (IND) ie, obvious hyperganglionosis in neuronal plexuses on PGP9.5 immunohistochemistry, ectopic ganglia in the mucosal and muscular layers on AchE histochemistry, and ghostlike ganglia on NADPH-diaphorase histochemistry were found. Likewise, in normal caliber distal colon from these mice, the distribution of ganglion cells, C-kit, and stem cell factor was normal. In control specimens, there was no ectopic ganglia or hyperganglionosis.<br />Conclusions: These findings suggest that the Ncx/Hox11L.1 gene is required for the proper innervation of the enteric nervous system in mice, and our deficient strain may be useful as a model for studying IND in humans.<br /> (Copyright 2001 by W.B. Saunders Company.)
- Subjects :
- Animals
Colonic Diseases pathology
Culture Techniques
Disease Models, Animal
Ganglia pathology
Ganglia physiology
Immunohistochemistry
Mice
Mice, Inbred C57BL
Mice, Inbred DBA
Mice, Transgenic
Neuropeptide Y analysis
Reference Values
Sensitivity and Specificity
Colon innervation
Colon pathology
Colonic Diseases genetics
Enteric Nervous System pathology
Gastrointestinal Motility genetics
Homeodomain Proteins genetics
Oncogene Proteins genetics
Subjects
Details
- Language :
- English
- ISSN :
- 0022-3468
- Volume :
- 36
- Issue :
- 8
- Database :
- MEDLINE
- Journal :
- Journal of pediatric surgery
- Publication Type :
- Academic Journal
- Accession number :
- 11479879
- Full Text :
- https://doi.org/10.1053/jpsu.2001.25797