Changes of Glycoconjugates in Hirschsprung's disease

1999 Fiscal Year Final Research Report Summary

• Project/Area Number: 09671837
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Single-year Grants
• Section: 一般
• Research Field: 小児外科
• Research Institution: Kyorin University
• Principal Investigator: ITO Yasuo Kyorin Univ., Pediatric Surgery, Professor, 医学部, 教授 (80110881)
• Co-Investigator(Kenkyū-buntansha): KAWAKAMI Hayato Kyorin Univ., Anatomy, Associate Professor, 医学部, 助教授 (30146542) ; HIRANO Hiroshi Kyorin Univ., Anatomy, Professor, 医学部, 教授 (10086481) ; SEKI Nobuo Kyorin Univ., Pediatric Surgery, Assistant, 医学部, 助手 (20281339)
• Project Period (FY): 1997 – 1999
• Keywords: Glycoconjugate / Hirschsprung's disease / Lectin

Research Abstract

Hirshsprung's disease is known to be caused by the absence of myenteric ganglia. We attempted to study the embryonic microenvironment which is mainly constituted by glycoconjugates and play an important role for undifferenciated neuroblasts to migrate and maturate. We applied lectin staining to observe the changes of glyoconjugates in the colon during development and in the colon of Hirschsprung's disease.
The lectin binding patterns in Auerbach's plexus of the distal portions of the rat colon from 15- to 21-day-old fetuses, newborns, and adults were examined by light and electron microscopy using sixteen different lectins (ConA, RCA-1, WGA, PNA, SBA, UEA-1, DBA, LCA, PHA-L, DSA, GS-1,VVA, MPA, BPA, MAA and PSA ). ConA was shown to increase in their binding after day 19 of gestation with differenciation of Auerbach's plexus, while DSA, MPA, and RCA-1 increased their staining intensity after day 17 of gestation in accordance with the appearance of Auerbach's Plexus. Under electron microscopy, DSA binding sites were localized mainly in the plasma membrane, Golgi apparatus, and nuclear membrane of nerve cells. Positivities were also observed in the axolemma and in the plasma membrane of nerve cell processus, Schwann cells and their surrounding smooth muscle cells. N-glycanase digestion test suggested that N-liked oligosaccbarides play an important role in the development of the Auerbach's plexus.
In Hirschsprung's disease, DSA, RCA-1 and WGA were stained stronger in the smooth muscle layer of the aganglionic segment than in that of the normo-ganglionic segment. This finding suggested that N-linked oligosaccharides may have been abnormal at the developmental stage of Auerbach's plexus in Hirschsprung's disease.