title: Expression analysis of BMP2, BMP5, BMP10 in human colon tissues from Hirschsprung disease patients.
abstract: OBJECTIVE
  Bone morphogenetic proteins (BMPs) are members of the transforming growth factor β (TGF β) superfamily. BMP2, BMP5 and BMP10 exert their biological functions by interacting with membrane bound receptors belonging to the serine/threonine kinase family. Hirschsprung disease (HSCR) is characterized by the absence of intramural ganglion cells in the nerve plexuses of the distal gut. However, putative Notch function in enteric nervous system (ENS) development and the etiology of HSCR is unknown.
  
  
  METHODS
  Aganglionic and ganglionic colon segment tissues of 50 HSCR patients were investigated for the expression pattern of BMP2, BMP5 and BMP10 using real-time RT-PCR, Western blot analysis and immunohistochemical staining.
  
  
  RESULTS
  The mRNA levels of BMP2, BMP5 and BMP10 in the stenotic colon segment from HSCR patients were significantly higher than those in the normal ones. Similar increased expressions of them in the stenotic colon segments were detected by Western blotting coupled with densitometry analysis. Lastly, immunohistologicl stain showed significant BMP2, 5 and 10 increases in mucous and muscular layers from stenotic colon segments compared to normal segments.
  
  
  CONCLUSIONS
  BMP2, BMP5 and BMP10 are elevated in the stenotic colon segment of HSCR, and BMPs signaling plays a pivotal role in the development of HSCR.
authors.affiliation: Key Laboratory of Health Ministry for Congenital Malformations, Shengjing Hospital of China Medical University Shenyang, Liaoning, People's Republic of China.
authors.forename: Mei
authors.initials: M
authors.lastname: Wu
authors.affiliation: Imaging and Nuclear Medicine, Shengjing Hospital of China Medical University 36 Sanhao Street, Shenyang, Liaoning, 110004, The People's Republic of China.
authors.forename: Wenwen
authors.initials: W
authors.lastname: Chen
authors.affiliation: Key Laboratory of Health Ministry for Congenital Malformations, Shengjing Hospital of China Medical University Shenyang, Liaoning, People's Republic of China.
authors.forename: Jie
authors.initials: J
authors.lastname: Mi
authors.affiliation: Key Laboratory of Health Ministry for Congenital Malformations, Shengjing Hospital of China Medical University Shenyang, Liaoning, People's Republic of China.
authors.forename: Dong
authors.initials: D
authors.lastname: Chen
authors.affiliation: Pediatric Surgery, Shengjing Hospital of China Medical University 36 Sanhao Street, Shenyang, Liaoning, 110004, The People's Republic of China.
authors.forename: Weilin
authors.initials: W
authors.lastname: Wang
authors.affiliation: Key Laboratory of Health Ministry for Congenital Malformations, Shengjing Hospital of China Medical University Shenyang, Liaoning, People's Republic of China.
authors.forename: Hong
authors.initials: H
authors.lastname: Gao
mesh_terms: D015153:Blotting, Western; D055396:Bone Morphogenetic Protein 2 / Q000032:analysis* / Q000235:genetics; D055417:Bone Morphogenetic Protein 5 / Q000032:analysis* / Q000235:genetics; D019485:Bone Morphogenetic Proteins / Q000032:analysis* / Q000235:genetics; D002675:Child, Preschool; D003106:Colon / Q000737:chemistry* / Q000473:pathology; D005260:Female; D006627:Hirschsprung Disease / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D007413:Intestinal Mucosa / Q000737:chemistry* / Q000473:pathology; D008297:Male; D012333:RNA, Messenger / Q000032:analysis; D060888:Real-Time Polymerase Chain Reaction; D020133:Reverse Transcriptase Polymerase Chain Reaction; D015398:Signal Transduction; D015854:Up-Regulation
chemical_list: C541386:BMP10 protein, human; C525718:BMP2 protein, human; C525814:BMP5 protein, human; D055396:Bone Morphogenetic Protein 2; D055417:Bone Morphogenetic Protein 5; D019485:Bone Morphogenetic Proteins; D012333:RNA, Messenger
keywords: Bone morphogenetic proteins (BMPs); Hirschsprung disease (HSCR); enteric nervous system (ENS); ganglion cell; stenotic colon