title: Smooth muscle proteins from Hirschsprung's disease facilitates stem cell differentiation.
abstract: OBJECTIVE
  The transplantation of neural crest derived stem cells (NCSC's) is a potent alternative for the treatment of Hirschsprung's disease (HSCR). Cells to be transplanted should find an appropriate microenvironment to survive and differentiate. To investigate the quality of this microenvironment, effects of HSCR-smooth-muscle-protein extracts upon NCSC's were studied in vitro.
  
  
  METHODS
  Postnatal human gut from children undergoing colonic resection due to HSCR was divided in segments. Smooth muscle was dissected and homogenized. Glial-cell-line-derived-neurotrophic-factor (GDNF) concentration was measured in the homogenates from the individual segment using ELISA. NCSC's were exposed to protein extracts derived from ganglionic and aganglionic HSCR segments, and their effect upon neurite outgrowth, survival and branching was evaluated.
  
  
  RESULTS
  The amount of the factors varied considerably between the proximal and distal segments, and also from patient to patient. While extracts from proximal segments tended to have more prominent effects, all HSCR-muscle-protein extracts increased neuronal survival and network formation.
  
  
  CONCLUSIONS
  Muscle protein from aganglionic bowel supports the survival and outgrowth of NCSC's and is so an appropriate target for neural stem cell treatment.
authors: Department of Pediatric Surgery, Medical Faculty Mannheim, University of Heidelberg, Theodor-Kutzer-Ufer 1-3, 68167, Mannheim, Germany.
authors: Cornelia Irene
authors: CI
authors: Hagl
authors: Sabine
authors: S
authors: Heumüller
authors: Markus
authors: M
authors: Klotz
authors: Ulrike
authors: U
authors: Subotic
authors: Lucas
authors: L
authors: Wessel
authors: Karl-Herbert
authors: KH
authors: Schäfer
mesh_terms: D002454:Cell Differentiation; D002675:Child, Preschool; D003082:Colectomy; D003106:Colon / Q000378:metabolism / Q000473:pathology*; D004797:Enzyme-Linked Immunosorbent Assay; D005786:Gene Expression Regulation; D006627:Hirschsprung Disease / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D007231:Infant, Newborn; D009124:Muscle Proteins / Q000235:genetics / Q000378:metabolism*; D009130:Muscle, Smooth / Q000378:metabolism*; D012333:RNA, Messenger / Q000235:genetics; D033581:Stem Cell Transplantation / Q000379:methods; D013234:Stem Cells / Q000166:cytology* / Q000378:metabolism
chemical_list: D009124:Muscle Proteins; D012333:RNA, Messenger