title: Comparative proteomic profiles of the normal and aganglionic hindgut in human Hirschsprung disease.
abstract: BACKGROUND
  Hirschsprung disease (HSCR) is the third most common congenital disorder of the gastrointestinal tract. This study aims to elucidate changes in protein expression between the normal and aganglionic hindgut in human HSCR.
  
  
  METHODS
  The biopsies were obtained from the normal and aganglionic hindgut in human HSCR, and the comparative proteomics were analyzed by mass spectrometry (MS)-based two-dimensional gel electrophoresis (2DE).
  
  
  RESULTS
  A total of 932-986 protein spots were identified in each of the gut segments, among which 30 spots had at least an eightfold difference in volume (%). Of the 30 differentially expressed spots, 15 proteins were identified via sequence analysis. Among these 15 proteins, eight were upregulated and seven were downregulated in the aganglionic group. The well-represented classes included biomarkers of enteric ganglions, extracellular matrix proteins, LIM domain proteins, serum proteins, and other pleiotropic proteins. Five proteins were selected and verified by western blotting and real-time PCR, and the results were consistent with the results of 2DE.
  
  
  CONCLUSIONS
  MS-based 2DE can help to identify pathological relevant proteins in HSCR; it defines an extensive protein catalog of the normal and aganglionic hindgut and may constitute the basis to understand pathophysiological mechanisms related to the HSCR.
authors.affiliation: Department of Pediatric Surgery, Major Laboratory of Chinese Health Ministry for Congenital Malformations, Shengjing Hospital of China Medical University, Shenyang, China.
authors.forename: Shu-Cheng
authors.initials: SC
authors.lastname: Zhang
authors.affiliation: Department of Hematology, Shengjing Hospital of China Medical University, Shenyang, China.
authors.forename: Fang
authors.initials: F
authors.lastname: Chen
authors.affiliation: Department of Pediatric Surgery, Major Laboratory of Chinese Health Ministry for Congenital Malformations, Shengjing Hospital of China Medical University, Shenyang, China.
authors.forename: Kai-Lei
authors.initials: KL
authors.lastname: Jiang
authors.affiliation: Department of Pediatric Surgery, Major Laboratory of Chinese Health Ministry for Congenital Malformations, Shengjing Hospital of China Medical University, Shenyang, China.
authors.forename: Zheng-Wei
authors.initials: ZW
authors.lastname: Yuan
authors.affiliation: Department of Pediatric Surgery, Major Laboratory of Chinese Health Ministry for Congenital Malformations, Shengjing Hospital of China Medical University, Shenyang, China.
authors.forename: Wei-Lin
authors.initials: WL
authors.lastname: Wang
mesh_terms: D015153:Blotting, Western; D015180:Electrophoresis, Gel, Two-Dimensional / Q000379:methods; D020869:Gene Expression Profiling; D006627:Hirschsprung Disease / Q000235:genetics* / Q000378:metabolism*; D006801:Humans; D013058:Mass Spectrometry / Q000379:methods; D020543:Proteome / Q000235:genetics / Q000378:metabolism*; D040901:Proteomics / Q000379:methods*; D060888:Real-Time Polymerase Chain Reaction; D017422:Sequence Analysis, DNA
chemical_list: D020543:Proteome