title: Differential expression of FOXA1, DUSP6, and HA117 in colon segments of Hirschsprung's disease.
abstract: OBJECTIVE
  To describe the expression profiles of FOXA1, DUSP6, and HA117 in different portions of the colon of patients diagnosed with Hirschsprung's disease (HSCR).
  
  
  METHODS
  Colon specimens were collected from 34 HSCR patients and grouped into 3 segments: proximal anastomosis, dilated segment and stenotic segment. Levels of FOXA1, DUSP6, and HA117 RNA were evaluated by real-time PCR. Levels of FOXA1 and DUSP6 protein were analyzed by immunohistochemistry and Western blotting.
  
  
  RESULTS
  The levels of FOXA1 and DUSP6 RNA were significantly lower in the stenotic segment compared to proximal anastomosis (P < 0.05). The level of HA117 RNA was significantly higher in the stenotic segment compared to proximal anastomosis (P < 0.05). In proximal anastomosis, FOXA1 and DUSP6 were both expressed at the protein level in ganglion cells and nerve fibers between the circular and longitudinal muscles. In the stenotic segments, positive staining for FOXA1 and DUSP6 was diminished. The levels of FOXA1 and DUSP6 protein were significantly lower in the stenotic segment compared to proximal anastomosis (P < 0.05).
  
  
  CONCLUSIONS
  Suppression of the FOXA1/DUSP6 signaling pathway may contribute to the development of HSCR. LncRNA HA117 may have an anti-differentiation function, and play a pivotal role in the progression of HSCR.
authors.affiliation: Ministry of Education Key Laboratory of Child Development and Disorders; Key Laboratory of Pediatrics in Chongqing; Chongqing International Science and Technology Cooperation Center for Child Development and Disorders, Children's Hospital of Chongqing Medical University Yuzhong District, Chongqing 400014, P. R. China ; Department of Neonatal Gastrointestinal Surgery, Children's Hospital of Chongqing Medical University Yuzhong District, Chongqing 400014, P. R. China.
authors.forename: Yuanyuan
authors.initials: Y
authors.lastname: Luo
authors.affiliation: Ministry of Education Key Laboratory of Child Development and Disorders; Key Laboratory of Pediatrics in Chongqing; Chongqing International Science and Technology Cooperation Center for Child Development and Disorders, Children's Hospital of Chongqing Medical University Yuzhong District, Chongqing 400014, P. R. China ; Department of Neonatal Gastrointestinal Surgery, Children's Hospital of Chongqing Medical University Yuzhong District, Chongqing 400014, P. R. China.
authors.forename: Shuangshuang
authors.initials: S
authors.lastname: Li
authors.affiliation: Ministry of Education Key Laboratory of Child Development and Disorders; Key Laboratory of Pediatrics in Chongqing; Chongqing International Science and Technology Cooperation Center for Child Development and Disorders, Children's Hospital of Chongqing Medical University Yuzhong District, Chongqing 400014, P. R. China ; Department of Neonatal Gastrointestinal Surgery, Children's Hospital of Chongqing Medical University Yuzhong District, Chongqing 400014, P. R. China.
authors.forename: Yinping
authors.initials: Y
authors.lastname: Teng
authors.affiliation: Ministry of Education Key Laboratory of Child Development and Disorders; Key Laboratory of Pediatrics in Chongqing; Chongqing International Science and Technology Cooperation Center for Child Development and Disorders, Children's Hospital of Chongqing Medical University Yuzhong District, Chongqing 400014, P. R. China ; Department of Neonatal Gastrointestinal Surgery, Children's Hospital of Chongqing Medical University Yuzhong District, Chongqing 400014, P. R. China.
authors.forename: Ning
authors.initials: N
authors.lastname: Wang
authors.affiliation: Ministry of Education Key Laboratory of Child Development and Disorders; Key Laboratory of Pediatrics in Chongqing; Chongqing International Science and Technology Cooperation Center for Child Development and Disorders, Children's Hospital of Chongqing Medical University Yuzhong District, Chongqing 400014, P. R. China ; Department of Neonatal Gastrointestinal Surgery, Children's Hospital of Chongqing Medical University Yuzhong District, Chongqing 400014, P. R. China.
authors.forename: Li
authors.initials: L
authors.lastname: Li
authors.affiliation: Ministry of Education Key Laboratory of Child Development and Disorders; Key Laboratory of Pediatrics in Chongqing; Chongqing International Science and Technology Cooperation Center for Child Development and Disorders, Children's Hospital of Chongqing Medical University Yuzhong District, Chongqing 400014, P. R. China ; Department of Neonatal Gastrointestinal Surgery, Children's Hospital of Chongqing Medical University Yuzhong District, Chongqing 400014, P. R. China.
authors.forename: Hang
authors.initials: H
authors.lastname: Liu
authors.affiliation: Ministry of Education Key Laboratory of Child Development and Disorders; Key Laboratory of Pediatrics in Chongqing; Chongqing International Science and Technology Cooperation Center for Child Development and Disorders, Children's Hospital of Chongqing Medical University Yuzhong District, Chongqing 400014, P. R. China ; Department of Neonatal Gastrointestinal Surgery, Children's Hospital of Chongqing Medical University Yuzhong District, Chongqing 400014, P. R. China.
authors.forename: Xianqing
authors.initials: X
authors.lastname: Jin
mesh_terms: D002675:Child, Preschool; D003106:Colon / Q000378:metabolism* / Q000473:pathology; D054642:Dual Specificity Phosphatase 6 / Q000378:metabolism*; D005260:Female; D005786:Gene Expression Regulation; D051541:Hepatocyte Nuclear Factor 3-alpha / Q000378:metabolism*; D006627:Hirschsprung Disease / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009363:Neoplasm Proteins / Q000235:genetics / Q000378:metabolism*; D015398:Signal Transduction
chemical_list: C496499:FOXA1 protein, human; C586650:HA117 protein, human; D051541:Hepatocyte Nuclear Factor 3-alpha; D009363:Neoplasm Proteins; C517035:DUSP6 protein, human; D054642:Dual Specificity Phosphatase 6
keywords: DUSP6; FOXA1; HA117; Hirschsprung’s disease