The down-regulation of neuroligin-2 and the correlative clinical significance of serum GABA over-expression in Hirschsprung's disease.
The goal of this study was to investigate the expression level of neuroligin-2 in different colon tissue segments of children with Hirschsprung's disease (HSCR) and the correlative clinical significance of serum Gamma-Aminobutyric Acid (serum GABA) in HSCR. Neuroligin-2 was assessed by Immunohistochemistry staining method on routine paraffin section from different colon tissue segments of HSCR (ganglionic colonic segment, transitional colonic segment and aganglionic colonic segment). Western-blot analysis and real-time fluorescence quantitative PCR(qRT-PCR) were applied to compare and evaluate the expression levels of neuroligin-2 from three segments of HSCR, and we used Enzyme-linked Immunosorbent Assay (ELISA) method to detect and compare the serum GABA between HSCR and non-HSCR. Immunohistochemistry staining demonstrated that intensive neuroligin-2 staining was detected in the ganglion cells in the ganglionic colonic and transitional colonic segments from the HSCR children; however, neuroligin-2 staining was down-regulated significantly in the aganglionic colonic segments. The expression levels of neuroligin-2 mRNA and protein in the aganglionic colonic segment were decreased compared to the ganglionic colonic segment and transitional colonic segment (P < 0.05). And the level of serum GABA was significantly higher in HSCR than that in non-HSCR. The expression of neuroligin-2 varies from different segments of HSCR. The down-regulation of neuroligin-2 in aganglionic colonic segments may be correlated with the excessive intestine contraction and further result in HSCR. The over-expression of serum GABA may be considered as a new diagnostic method of HSCR.
Department of Pediatric Surgery, Qilu Hospital, Shandong University, 44#, Wenhua Xi Road, Jinan, 250012, Shandong, People's Republic of China.
Hongchao
H
Yang
Jianyi
J
Niu
Jian
J
Wang
Fan
F
Zhang
Qiangye
Q
Zhang
Wentong
W
Zhang
Aiwu
A
Li
D015415:Biomarkers / Q000097:blood / Q000378:metabolism; D015816:Cell Adhesion Molecules, Neuronal / Q000378:metabolism*; D002675:Child, Preschool; D015536:Down-Regulation / Q000502:physiology*; D005260:Female; D005786:Gene Expression Regulation; D006627:Hirschsprung Disease / Q000097:blood / Q000175:diagnosis / Q000378:metabolism*; D006801:Humans; D007223:Infant; D007413:Intestinal Mucosa / Q000378:metabolism* / Q000473:pathology; D008297:Male; D009419:Nerve Tissue Proteins / Q000378:metabolism*; D005680:gamma-Aminobutyric Acid / Q000096:biosynthesis / Q000097:blood*
D015415:Biomarkers; D015816:Cell Adhesion Molecules, Neuronal; D009419:Nerve Tissue Proteins; C098003:neuroligin 2; D005680:gamma-Aminobutyric Acid