title: Involvement of down-regulated E2F3 in Hirschsprung's disease.
abstract: OBJECTIVE
  Hirschsprung's disease (HSCR) is a common cause of neonatal bowel obstruction characterized by the absence of ganglion cells in the colon. Impaired migration of the neural crest cells (NCCs) has been implicated as one of the main causes of HSCR. E2F3, a member in the E2F family, which plays a crucial role in the control of the cell cycle is correlated with neuron migration. However, the function of E2F3 in the development of the enteric nervous system still remains unknown. This study aims to reveal the correlation of E2F3 in the progress of HSCR.
  
  
  METHODS
  By using reverse transcriptase polymerase chain reaction (RT-PCR) and western blot assay, we investigated levels of E2F3 expression in 58 HSCR patients, both in the aganglionic bowel segment and the normal ganglionic segment, and in 39 unrelated controls. By in vitro assays, we used the siRNA method to knock-down the level of E2F3 expression in 293T cell lines. Furthermore, transwell assay was used to detect cell migration ability.
  
  
  RESULTS
  Aberrant lower expression level of E2F3 was detected in the HSCR-S segment compared with the control group by RT-PCR and western blot assay. Besides, down-regulated E2F3 could suppress the cell migration.
  
  
  CONCLUSIONS
  This is the first study showing the down-regulation of E2F3 in HSCR, bringing new insight to the mechanism of the impaired migration of neural crest cells.
authors.affiliation: State Key Laboratory of Reproductive Medicine, Institute of Toxicology, School of Public Health, Nanjing Medical University, Nanjing, China.
authors.forename: Weibing
authors.initials: W
authors.lastname: Tang
authors.forename: Junwei
authors.initials: J
authors.lastname: Tang
authors.forename: Jingjing
authors.initials: J
authors.lastname: Qin
authors.forename: Qiming
authors.initials: Q
authors.lastname: Geng
authors.forename: Zhigang
authors.initials: Z
authors.lastname: Zhou
authors.forename: Bo
authors.initials: B
authors.lastname: Li
authors.forename: Jie
authors.initials: J
authors.lastname: Zhang
authors.forename: Huan
authors.initials: H
authors.lastname: Chen
authors.forename: Yankai
authors.initials: Y
authors.lastname: Xia
authors.forename: Xinru
authors.initials: X
authors.lastname: Wang
mesh_terms: D015153:Blotting, Western; D002460:Cell Line; D002465:Cell Movement; D002648:Child; D015536:Down-Regulation; D050699:E2F3 Transcription Factor / Q000378:metabolism*; D005260:Female; D006627:Hirschsprung Disease / Q000378:metabolism* / Q000601:surgery; D006801:Humans; D008297:Male; D009432:Neural Crest / Q000166:cytology; D020133:Reverse Transcriptase Polymerase Chain Reaction; D018709:Statistics, Nonparametric
chemical_list: D050699:E2F3 Transcription Factor; C098319:E2F3 protein, human