Proteomic analysis of differentially expressed proteins between stenotic and normal colon segment tissues derived from patients with Hirschsprung's disease.

Hirschsprung's disease (HSCR) is the most common identifiable developmental disorder of the enteric nervous system. The present study was designed to analyze the differential proteomic patterns in stenotic colon segment tissues from patients with HSCR. We analyzed 20 paired stenotic and normal colon segment tissues from patients with HSCR, and identified 13 proteins from stenotic segment tissues peptide fingerprint mapping and SELDI MS that were separated using 2-DE. The protein levels of four selected proteins (α-actinin-4, ACTN4; myosin regulatory light chain interacting protein, MYLIP; fatty acid binding protein 7, FABP7; bone morphogenetic protein receptor type 1A, BMPR1A) were further validated by Western blot analysis. This study, investigating for the first time proteomic changes in stenotic colon segment tissues from patients with HSCR, provides potential markers or promising new candidate actors for the pathogenesis of HSCR.