title: Expression and significance of neuroligins in myenteric cells of Cajal in Hirschsprung's disease.
abstract: OBJECTIVE
  The aim of this study was to investigate the expression and significance of neuroligins in myenteric cells of Cajal (ICC-MY) in Hirschsprung's disease (HSCR).
  
  
  METHODS
  Longitudinal muscle with adherent myenteric plexus (LMMP) from surgical excision waste colon of HSCR children were prepared by peeling off the mucous layer, sub-mucosal layer and circular muscle. Neuroligins, c-Kit (c-Kit-immunoreactivity representing ICC) and their relationship were assessed by double labeling immunofluorescence staining. ICC-MY were dissociated and cultured from LMMP by enzymolysis method, and were purified and analyzed using a combination of magnetic-activated cell sorting (MACS) and flow cytometry (FCM). Western-blot analysis was applied to compare and evaluate the expression levels of neuroligins in ICC-MY which were dissociated from different segments of HSCR (ganglionic colonic segment, transitional colonic segment and aganglionic colonic segment).
  
  
  RESULTS
  Neuroligins and c-Kit were expressed on the same cells (ICC-MY); ICC-MY were dissociated, cultured and purified. For HSCR, neuroligins were expressed significantly in ICC-MY from ganglionic colonic segments, moderately in those from transitional colonic segments and down-regulated significantly in those from aganglionic colonic segments.
  
  
  CONCLUSIONS
  Neuroligins were expressed in ICC-MY of human beings, and the expression varies from different segments of HSCR. This abnormal expression might play an important role in the pathogenesis of this disease through affecting the synaptic function of ICC-MY.
authors.affiliation: Department of Pediatric Surgery, Qilu Hospital, Shandong University, Jinan, Shandong, China.
authors.forename: Jian
authors.initials: J
authors.lastname: Wang
authors.forename: Yaru
authors.initials: Y
authors.lastname: Mou
authors.forename: Qiangye
authors.initials: Q
authors.lastname: Zhang
authors.forename: Fan
authors.initials: F
authors.lastname: Zhang
authors.forename: Hongchao
authors.initials: H
authors.lastname: Yang
authors.forename: Wentong
authors.initials: W
authors.lastname: Zhang
authors.forename: Aiwu
authors.initials: A
authors.lastname: Li
mesh_terms: D015816:Cell Adhesion Molecules, Neuronal / Q000378:metabolism*; D002675:Child, Preschool; D003106:Colon / Q000473:pathology / Q000601:surgery; D005260:Female; D006627:Hirschsprung Disease / Q000378:metabolism* / Q000473:pathology / Q000601:surgery; D006801:Humans; D007223:Infant; D056885:Interstitial Cells of Cajal / Q000378:metabolism*; D008297:Male; D008565:Membrane Proteins / Q000378:metabolism; D009130:Muscle, Smooth / Q000473:pathology; D009197:Myenteric Plexus / Q000473:pathology*; D009419:Nerve Tissue Proteins / Q000378:metabolism
chemical_list: D015816:Cell Adhesion Molecules, Neuronal; D008565:Membrane Proteins; C430845:NLGN4X protein, human; D009419:Nerve Tissue Proteins; C093438:neuroligin 1; C098003:neuroligin 2; C098004:neuroligin 3