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. CONCLUSION: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.

journal_name

Pediatr Res

journal_title

Pediatric research

authors

Zhang SC,Chen F,Jiang KL,Yuan ZW,Wang WL

doi

10.1038/pr.2014.33

subject

Has Abstract

pub_date

2014-06-01 00:00:00

pages

754-61

issue

6

eissn

0031-3998

issn

1530-0447

pii

pr201433

journal_volume

75

pub_type

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