Localization of intestinal pacemaker cells and synapses in the muscle layers of a patient with colonic hypoganglionosis

• Published in: Journal of pediatric surgery Vol. 31; no. 4; pp. 584 - 587
• Main Authors: Yamataka, A., Fujiwara, T., Nishiye, H., Sunagawa, M., Miyano, T.
• Format: Journal Article Conference Proceeding
• Language: English
• Published: Philadelphia, PA Elsevier Inc 01-04-1996; Elsevier
• Subjects: Biological and medical sciences; Colon - innervation; Colon - pathology; Fluorescent Antibody Technique, Indirect; Follow-Up Studies; Gastroenterology. Liver. Pancreas. Abdomen; Gastrointestinal Motility - physiology; Hirschsprung Disease - pathology; Hirschsprung Disease - surgery; Humans; Ileostomy; Ileum - innervation; Ileum - pathology; Infant; Infant, Newborn; Male; Malformations; Medical sciences; Myenteric Plexus - pathology; Proto-Oncogene Proteins c-kit - analysis; Stomach. Duodenum. Small intestine. Colon. Rectum. Anus; Synapses - pathology; Synaptic Transmission - physiology; Hypoganglionosis; intestinal pacemaker; c- kit; immunohistochemistry
• ISSN: 0022-3468; 1531-5037
• DOI: 10.1016/S0022-3468(96)90502-3

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 muscles 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.