| 【Objective】To investigate the expression levels of β-1,4-galactosyltransferase-Ⅰand detect the locations changes of the total galactosylated of glycoproteins(β-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 β-1,4-galactosyltransferase-Ⅰin 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 ofβ-1,4-galactosyltransferase-Ⅰ in aganglionic segment showed a significant increase when they were compared to the normal segment.There were no significant differences in theβ-1,4-galactosyltransferase-Ⅰ 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】β-1,4-galactosyltransferase-Ⅰ in the congenital megacolon stenosis(no ganglion cell segment),transitional segment,expansion of the expression decreased in turn.There is a positive correlation between β-1,4-galactosyltransferase-Ⅰand laminin,both with the occurrence of congenital megacolon may exist some kind of internal link. |
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