Expression levels of smoothelin in colonic tissues from patients with Hirschsprung’s disease

Abstract

Objective To explore the expression level of smoothelin (SM) protein and mRNA in Hirschsprung's disease (HD) and elucidate the relationship between the expression of SM and the pathogenesis of HD. Methods Full-thickness colonic specimens were obtained from 53 HD patients undergoing pull-through procedure between January 2007 and January 2013.There were 33 boys and 20 girls with an age range of 5 months to 4 years.The diagnosis of HD was con? rmed jointly by hematoxylin & eosin histochemistry, PGP 9.5, neuron specific enolase (NSE) and S100 immunohistochemistry.Quantitative reverse transcription-polymerase chain reaction (RT-PCR) and Western blot were used for detecting SM mRNA and protein expression in different segments of colonic tissue. Results The expression levels of SM mRNA and protein were 27.13±16.44×10-3, 21.60±10.02×10-3, 19.66±9.92×10-3 and 43.13±12.44, 36.83±9.43, 23.63±4.97 in ganglionic, transitional and aganglionic segments respectively.Both mRNA and protein of SM were markedly less in transitional and aganglionic segments than those in ganglionic segment (P<0.05). Conclusions SM may be involved in the pathogenesis of HD. Key words: Hirschsprung's disease; Western blot; Reverse transcription-polymerase chain reaction