Comparative study of Hsp27, GSK3β, Wnt1 and PRDX3 in Hirschsprung's disease.
Hirschsprung's disease (HSCR) is a developmental disorder of the enteric nervous system characterized by aganglionosis in distal gut. In this study, we used two-dimensional gel electrophoresis (2-DE) technology coupled with matrix assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF-MS) analysis to identify differentially expressed proteins in the aganglionic (stenotic) and ganglionic (normal) colon segment tissues from patients with HSCR. We identified 15 proteins with different expression levels between the stenotic and the normal colon segment tissues from patients with HSCR. Nine proteins were upregulated and six proteins downregulated in the stenotic colon segment tissues compared to the normal colon segment tissues. Based on the biological functions, we selected the Hsp27 upregulated proteins and the PRDX3 downregulated proteins to confirm their expression in 20 patients. The protein and mRNA expressions of Hsp27 were statistically higher in the stenotic colon segment tissues than in the normal colon segment tissues, whereas the protein and mRNA expressions of PRDX3 were statistically lower in the stenotic colon segment tissues than in the normal colon segment tissues. These findings of changes in mRNA and protein in tissues from patients with HSCR provide information which may be helpful in understanding the pathomechanism that is implicated in the disease.
Laboratory of Pediatric Congenital Malformation, Ministry of Public Health, Shengjing Hospital of China Medical University, Shenyang, China.
Hong
H
Gao
Xiaomei
X
Liu
Dong
D
Chen
Liangying
L
Lv
Mei
M
Wu
Jie
J
Mi
Weilin
W
Wang
D002681:China; D003106:Colon / Q000378:metabolism; D015180:Electrophoresis, Gel, Two-Dimensional; D005260:Female; D005786:Gene Expression Regulation; D038362:Glycogen Synthase Kinase 3 / Q000235:genetics / Q000378:metabolism*; D000071679:Glycogen Synthase Kinase 3 beta; D055551:HSP27 Heat-Shock Proteins / Q000235:genetics / Q000378:metabolism*; D006360:Heat-Shock Proteins; D006627:Hirschsprung Disease / Q000378:metabolism*; D006801:Humans; D007223:Infant; D008297:Male; D018832:Molecular Chaperones; D061105:Peroxiredoxin III / Q000235:genetics / Q000378:metabolism*; D040901:Proteomics; D012333:RNA, Messenger / Q000235:genetics; D019032:Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization; D051155:Wnt1 Protein / Q000235:genetics / Q000378:metabolism*
D055551:HSP27 Heat-Shock Proteins; C057725:HSPB1 protein, human; D006360:Heat-Shock Proteins; D018832:Molecular Chaperones; D012333:RNA, Messenger; C495247:WNT1 protein, human; D051155:Wnt1 Protein; C516115:PRDX3 protein, human; D061105:Peroxiredoxin III; C000605506:GSK3B protein, human; D000071679:Glycogen Synthase Kinase 3 beta; D038362:Glycogen Synthase Kinase 3
Hirschsprung's disease; Hsp27 and PRDX3; colon segment tissues; different expressions