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. 2001 Feb;7(1):16-21.
doi: 10.3748/wjg.v7.i1.16.

The development of colon innervation in trisomy 16 mice and Hirschsprung's disease

J C Li  1 , K H Mi, J L Zhou, L Busch, W Kuhnel
Affiliations

The development of colon innervation in trisomy 16 mice and Hirschsprung's disease

J C Li et al. World J Gastroenterol. 2001 Feb.

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 Hirschsprung's disease (HD).

Methods: Trisomy 16 mouse breeding;cytogenetic analysis of trisomy 16 mice; and PG P9.5 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 5mm 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 revealed 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.

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Figures

Figure 1
Figure 1
a. The chromosome of normal mice. b. The chromosome of trisomy 16 mice: two Rb metecentric chromosomes (↑) and 41 chromosome arms. × 1000
Figure 2
Figure 2
The survival rate of trisomy 16 mice with different embryonic days (ED).
Figure 3
Figure 3
a. Myenteric plexus of normal littermates at ED 15. The nerve meshworks had regular meshes with many neurons (↑). × 400 b. The colon of trisomy 16 mice contained some neurons (arrow) with different staining and size at ED15. × 400
Figure 4
Figure 4
At ED 18, the myenteric plexuses of trisomy 16 mice and their normal littermates in whole mount preparation. × 250 A. The developed myenteric plexus with primary (long arrow), secondary (arrow) and tertiary (arrowhead) strands were displayed in the colon of normal littermates. The primary strands were composed of broad fiber bundles and numerous ganglia with longitudinally arranged meshes. B. The myenteric plexus of trisomy 16 mice was poor with less ganglia containing some neurons (arrow) at different staining.
Figure 5
Figure 5
At ED 18, the nerve fibers (long arrow) stretching from the enteric plexuses reached the gut villi (Vi). The myenteric (arrowhead) and submucosal (arrow) plexuses were clear. × 160
Figure 6
Figure 6
Aganglionic segment of trisomy 16 mouse colon. × 160 B. The colon of normal littermate with ganglia (arrow). × 100
Figure 7
Figure 7
The myenteric nervous fibers (arrow) were detected in the colon (C) mesentery of trisomy 16 mice. × 160
Figure 8
Figure 8
The submucosal ganglia (arrow) of normal colon of human. S: submucosal muscular layer. × 100
Figure 9
Figure 9
The aganglionic colon of Hirschprung’s disease. Abundant proliferation of nerve fibers (large arrow) was found in the stenosed segment of colon. A wave-like nerve fiber (arrow). × 100
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References

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