OBJECTIVETo 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.

METHODSFrom 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.

RESULTSA 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.

CONCLUSIONNotch-1 and Jagged-2 are not expressed in spastic colon segments, which may be associated with the pathogenesis of HD.