International Journal of Colorectal Disease

, Volume 27, Issue 1, pp 37–41

Downregulation of Notch-1/Jagged-2 in human colon tissues from Hirschsprung disease patients

  • Huimin Jia
  • Keren Zhang
  • Qingjiang Chen
  • Hong Gao
  • Weilin Wang
Original Article

DOI: 10.1007/s00384-011-1295-4

Cite this article as:
Jia, H., Zhang, K., Chen, Q. et al. Int J Colorectal Dis (2012) 27: 37. doi:10.1007/s00384-011-1295-4

Abstract

Purpose

Recent studies have shown that the Notch pathways play important roles in the differentiation and development of neurons. Hirschsprung disease (HSCR) is characterized by the absence of intramural ganglion cells in the nerve plexuses of the distal gut. However, putative Notch function in enteric nervous system (ENS) development and the etiology of HSCR is unknown.

Materials and methods

The aganglionosis segments of 30 HSCR patients were introduced to investigate the expression pattern of Notch-1 and Jagged-2 using immunohistochemical staining, reverse transcriptase polymerase chain reaction (RT-PCR), and Western blot analysis.

Results

Intensive Notch-1 and Jagged-2 staining was detected in the submucosal and the myenteric plexuses in normal or oligoganglionosis segments. Aganglionosis segments from HSCR patients contained no plexuses and thus not labeled with Notch-1 and Jagged-2. Western blot analysis revealed reduced Notch-1 and Jagged-2 protein levels, and RT-PCR revealed reduced Notch-1 and Jagged-2 mRNA in the aganglionosis segments of HSCR patients.

Conclusions

This study is the first illustration of Notch-1 and Jagged-2 expression in human tissues from non-cancerous disease and sets up the base for further investigations of Notch function in ENS development and intestinal motility.

Keywords

Hirschsprung disease Notch Enteric nervous system 

Copyright information

© Springer-Verlag 2011

Authors and Affiliations

  • Huimin Jia
    • 1
  • Keren Zhang
    • 1
  • Qingjiang Chen
    • 1
  • Hong Gao
    • 2
  • Weilin Wang
    • 1
  1. 1.Department of Pediatric SurgeryShengjing Hospital, China Medical UniversityShenyangPeople’s Republic of China
  2. 2.The Key Laboratory of Health Ministry for Congenital MalformationShenyangPeople’s Republic of China

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