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 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.