[Expression and significance of Notch-1 and Jagged-2 in patients with Hirschsprung disease].
OBJECTIVE
To investigate the expression of Notch-1 and Jagged-2 in the normal and spastic segments of colon in patients with Hirschsprung disease(HD), and to explore the correlation of Notch-1 and Jagged-2 with pathogenesis of HD.


METHODS
From 2005 to 2010, resected colon specimens of 30 cases with HD were selected for this study. Normal colonic segments were served as control group, while the transitional and spastic segments as experimental group. Immunohistochemical staining, Western blotting, and RT-PCR were applied to detect the expression of Notch-1 and Jagged-2.


RESULTS
A large number of Notch-1 and Jagged-2 positive gangliocytes were observed in the control group, while none was observed in spastic segments. Significantly less Notch-1 and Jagged-2 positive gangliocytes were found in the transitional segments. Western blotting revealed that Notch-1 and Jagged-2 protein levels in spastic segments (0.19±0.02 and 0.13±0.04) were less than that in transitional segments and normal segments (0.58±0.05 and 0.52±0.04, 0.72±0.04 and 0.69±0.04, respectively)(P<0.05). RT-PCR revealed that Notch-1 and Jagged-2 mRNA levels were consistent with protein expression.


CONCLUSIONS
Notch-1 and Jagged-2 are not expressed in spastic colon segments, which may be associated with the pathogenesis of HD.
Department of Pediatric Surgery, Shengjing Hospital, China Medical University, Shenyang, China.
Hui-min
HM
Jia
Xiu-fang
XF
Han
Yu-zuo
YZ
Bai
Wei-lin
WL
Wang
D016022:Case-Control Studies; D005260:Female; D006627:Hirschsprung Disease / Q000235:genetics* / Q000378:metabolism; D006801:Humans; D007223:Infant; D036341:Intercellular Signaling Peptides and Proteins / Q000235:genetics*; D000072101:Jagged-2 Protein; D008297:Male; D008565:Membrane Proteins / Q000235:genetics*; D012333:RNA, Messenger / Q000235:genetics; D051881:Receptor, Notch1 / Q000235:genetics*; D020133:Reverse Transcriptase Polymerase Chain Reaction; D015398:Signal Transduction
D036341:Intercellular Signaling Peptides and Proteins; C490504:JAG2 protein, human; D000072101:Jagged-2 Protein; D008565:Membrane Proteins; C497761:NOTCH1 protein, human; D012333:RNA, Messenger; D051881:Receptor, Notch1