Anne-Marie O'Donnell1, David Coyle1, Prem Puri1. 1. Anne-Marie O'Donnell, David Coyle, Prem Puri, National Children's Research Centre, Our Lady's Children's Hospital Crumlin, 12 Dublin, Ireland.
Abstract
AIM: 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. CONCLUSION: 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.
AIM: 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. CONCLUSION: 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: Masaaki Kurahashi; Haifeng Zheng; Laura Dwyer; Sean M Ward; Sang Don Koh; Kenton M Sanders Journal: J Physiol Date: 2010-12-20 Impact factor: 5.182
Authors: Salah A Baker; Grant W Hennig; Anna K Salter; Masaki Kurahashi; Sean M Ward; Kenton M Sanders Journal: J Physiol Date: 2013-10-21 Impact factor: 5.182