Original Articles
Copyright ©The Author(s) 2001. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Feb 15, 2001; 7(1): 16-21
Published online Feb 15, 2001. doi: 10.3748/wjg.v7.i1.16
The development of colon innervation in trisomy 16 mice and Hirschsprungs disease
Ji Cheng Li, Kai Hong Mi, Ji Lin Zhou, LC Busch, W Kuhnel
Ji Cheng Li, Kai Hong Mi, Ji Lin Zhou, Department of Lymphology, Department of Histology and Embryology, Zhejiang University Medical School, Hangzhou 310031, China
LC Busch, W Kuhnel, Institute of Antomy, Medical University of Lubeck, Germany
Ji Cheng Li, graduated from Zhejiang Medical University in 1983, now professor of histology and embryology, engaged in the research of Hirschsprung’s disease, having 50 papers published.
Author contributions: All authors contributed equally to the work.
Supported by the Grants of Analysis and Measurement of Zhejiang Province and Education Committee of Zhejiang Province.
Correspondence to: Dr. Ji Cheng Li, Department of Lymphology, Department of Histology and Embryology, Zhejiang University Medical School, Hangzhou 310031, Zhejiang Province, China. lijc@mail.hz.zj.cn
Telephone: 0086-571-7217139 Fax: 0086-571-8855930
Received: September 2, 2000
Revised: October 15, 2000
Accepted: October 22, 2000
Published online: February 15, 2001
Abstract

AIM: To study the colon innervation of trisomy 16 mouse, an animal model for Down’s syndrome, and the expression of protein gene product 9.5 (PGP 9.5) in the stenosed segment of colon in Hirschsprungs disease (HD).

METHODS: Trisomy 16 mouse breeding; cytogenetic analysis of trisomy 16 mice; and PGP 9.5 immunohistochemistry of colons of trisomy 16 mice and HD were carried out.

RESULTS: Compared with their normal littermates, the nervous system of colon in trisomy 16 mice was abnormally developed. There existed developmental delay of muscular plexuses of colon, no submucosal plexus was found in the colon, and there was 5 mm aganglionic bowel aparting from the anus in trisomy 16 mice. The mesentery nerve fibers were as well developed as shown in their normal littermates. Abundant proliferation of PGP 9.5 positive nerve fibers was evealed in the stenosed segment of HD colon.

CONCLUSION: Trisomy 16 mice could serve as an animal model for Hirschsprung’s disease for aganglionic bowel in the distal part of colon. Abundant proliferation of PGP 9.5 positive fibers resulted from extrinsic nerve compensation, since no ganglionic cells were observed in the stenosed segment of the colon in HD. HD has a genetic tendency.

Keywords: Hirschsprungs disease; colon; down syndrome; immunohistochemistry; nervous system; trisomy 16 mouse