| ?Objective?To investigate the expression levels of ?-1,4-galactosyltransferase-?and detect the locations changes of the total galactosylated of glycoproteins(?-1,4GlcNAc)and laminin in different segments of colonic tissues from patients with HD and analyze their potential pathogenic roles in Hirschsprung‘s disease.?Methods?The mRNAs and proteins expression of ?-1,4-galactosyltransferase-?in the aganglionic,transitional and normal segment in 37 cases with HD and 6 control samples from the children with Intussusception was assayed by real-time PCR and Western blot,respectively.Immunohistochemical staining was used to determine the expression and localization of Ricinus communis agglutinin(RCA-I)and laminin in the three segments tissues and control bowels.?Results?1?The mRNA and protein levels of?-1,4-galactosyltransferase-? in aganglionic segment showed a significant increase when they were compared to the normal segment.There were no significant differences in the?-1,4-galactosyltransferase-? expression between the normal ganglionic segment of HD patients and control bowels.2?Immunochemical staining indicates the expression of RCA-I and laminin was staining higher in aganglionic segment than those in normal control tissues.Moderate expression of RCA-I and laminin were observed in the migratory segment,which was significantly different from that in the ganglionic and aganglionic segments in patients with HD.The RCA-I and laminin expression in the normal ganglionic segment of HD patients and control bowels were no significant differences.3?The positive correlation between the expressions of RCA-I and laminin.?Conclusions??-1,4-galactosyltransferase-? in the congenital megacolon stenosis(no ganglion cell segment),transitional segment,expansion of the expression decreased in turn.There is a positive correlation between ?-1,4-galactosyltransferase-?and laminin,both with the occurrence of congenital megacolon may exist some kind of internal link. |
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