title: Localization of intestinal pacemaker cells and synapses in the muscle layers of a patient with colonic hypoganglionosis.
abstract: Bowel dysmotility in association with hypoganglionosis remains unexplained. The proto-oncogene c-kit encodes a transmembrane tyrosine kinase receptor, and the c-kit protein-product (C-KIT) positive cells in the mammalian gut are responsible for intestinal pacemaker activity. The authors examined the localization of intestinal pacemaker cells in the muscle layers of a patient with colonic hypoganglionosis, using an antihuman C-KIT serum. In the normoganglionic ileum, many C-KIT immunoreactivity positive (C-KIT-IR+) cells were present in the muscle layers. In contrast, there were no C-KIT-IR+ cells in the muscle layers of the hypoganglionic colon. These findings suggest that a lack of c-kit expression may be responsible for the autonomic gut dysmotility in hypoganglionic bowels.
authors.affiliation: Division of Pediatric Surgery, Dokkyo University School of Medicine, Tochigi, Japan.
authors.forename: A
authors.initials: A
authors.lastname: Yamataka
authors.forename: T
authors.initials: T
authors.lastname: Fujiwara
authors.forename: H
authors.initials: H
authors.lastname: Nishiye
authors.forename: M
authors.initials: M
authors.lastname: Sunagawa
authors.forename: T
authors.initials: T
authors.lastname: Miyano
mesh_terms: D003106:Colon / Q000294:innervation / Q000473:pathology; D019084:Fluorescent Antibody Technique, Indirect; D005500:Follow-Up Studies; D005769:Gastrointestinal Motility / Q000502:physiology*; D006627:Hirschsprung Disease / Q000473:pathology* / Q000601:surgery; D006801:Humans; D007081:Ileostomy; D007082:Ileum / Q000294:innervation / Q000473:pathology; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009197:Myenteric Plexus / Q000473:pathology; D000090063:Proto-Oncogene Mas; D019009:Proto-Oncogene Proteins c-kit / Q000032:analysis*; D013569:Synapses / Q000473:pathology*; D009435:Synaptic Transmission / Q000502:physiology*
chemical_list: C000715470:MAS1 protein, human; D000090063:Proto-Oncogene Mas; D019009:Proto-Oncogene Proteins c-kit