title: Deficiency of platelet-derived growth factor receptor-α-positive cells in Hirschsprung's disease colon.
abstract: OBJECTIVE
  To investigate whether the expression of platelet-derived growth factor receptor-α-positive (PDGFRα(+))-cells is altered in Hirschsprung's disease (HD).
  
  
  METHODS
  HD tissue specimens (n = 10) were collected at the time of pull-through surgery, while colonic control samples were obtained at the time of colostomy closure in patients with imperforate anus (n = 10). Immunolabelling of PDGFRα(+)-cells was visualized using confocal microscopy to assess the distribution of these cells, while Western blot analysis was undertaken to quantify PDGFRα protein expression.
  
  
  RESULTS
  Confocal microscopy revealed PDGFRα(+)-cells within the mucosa, myenteric plexus and smooth muscle in normal controls, with a marked reduction in PDGFRα(+)-cells in the HD specimens. Western blotting revealed high levels of PDGFRα protein expression in normal controls, while there was a striking decrease in PDGFRα protein expression in the HD colon.
  
  
  CONCLUSIONS
  These findings suggest that the altered distribution of PDGFRα(+)-cells in both the aganglionic and ganglionic HD bowel may contribute to the motility dysfunction in HD.
authors.affiliation: Anne-Marie O'Donnell, David Coyle, Prem Puri, National Children's Research Centre, Our Lady's Children's Hospital Crumlin, 12 Dublin, Ireland.
authors.forename: Anne-Marie
authors.initials: AM
authors.lastname: O'Donnell
authors.affiliation: Anne-Marie O'Donnell, David Coyle, Prem Puri, National Children's Research Centre, Our Lady's Children's Hospital Crumlin, 12 Dublin, Ireland.
authors.forename: David
authors.initials: D
authors.lastname: Coyle
authors.affiliation: Anne-Marie O'Donnell, David Coyle, Prem Puri, National Children's Research Centre, Our Lady's Children's Hospital Crumlin, 12 Dublin, Ireland.
authors.forename: Prem
authors.initials: P
authors.lastname: Puri
mesh_terms: D015153:Blotting, Western; D016022:Case-Control Studies; D003106:Colon / Q000737:chemistry* / Q000473:pathology / Q000503:physiopathology / Q000601:surgery; D015536:Down-Regulation; D005455:Fluorescent Antibody Technique; D005769:Gastrointestinal Motility; D006627:Hirschsprung Disease / Q000378:metabolism* / Q000473:pathology / Q000503:physiopathology / Q000601:surgery; D006801:Humans; D007223:Infant; D018613:Microscopy, Confocal; D020796:Receptor, Platelet-Derived Growth Factor alpha / Q000172:deficiency*
chemical_list: D020796:Receptor, Platelet-Derived Growth Factor alpha
keywords: Aganglionosis; Gastrointestinal motility; Hirschsprung’s disease; Myenteric plexus; Platelet-derived growth factor receptor alpha