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Article: Ret protein in the human fetal rectum

TitleRet protein in the human fetal rectum
Authors
Issue Date1996
PublisherWB Saunders Co. The Journal's web site is located at http://www.elsevier.com/locate/jpedsurg
Citation
Journal of Pediatric Surgery, 1996, v. 31 n. 4, p. 568-571 How to Cite?
AbstractA major gene for Hirschsprung's disease (HD) recently has been mapped in chromosome 10q11.2 and identified to be the RET proto-oncogene. Mutations of the RET gene have occurred in HD patients, and abnormalities of expression and function of Ret protein (a receptor tyrosine kinase, which is the product of the RET gene) have been found in their intestines. In vitro studies of the biological effects of HD mutations suggest a loss of function effect, which may be negative-dominant. However, the developmental role of the Ret protein in the organogenesis of the enteric nervous system (ENS) and its role in the pathogenesis of HD remain unclear. The authors present a study of the expression of Ret protein in the human ENS during fetal development. Fresh rectal tissues were obtained from nine fetuses (gestational age range, 12 to 22 weeks). Ret protein expression was studied immunohistochemically, using antibodies against the carboxy-terminal 20 amino acids (anti-Ret C) and the extracellular domain (anti-Ret R5). The tyrosine kinase activity of the fetal ENS was investigated with antiphosphotyrosine mouse monoclonal antibody against the phosphorylated tyrosine residues. Anti-Ret C immunostaining was observed in ganglion cells at all ages, but intense activity was significantly higher among the cells of the younger fetuses. Intense anti-Ret R5 immunostaining was present in the enteric ganglion cells of the 12-week-old fetus. The tyrosine kinase activity of ganglion cells increases progressively with advancing gestational age. The results of this study support the hypothesis that the Ret protein receptor might play a crucial role in the cellular and molecular processes involved in the development and maturation of the ENS, abnormalities of which could result in HD. High Ret protein expression and low tyrosine kinase activity have been reported to occur in small ganglia of the HD hypoganglionic segment. In the present study, these markers were typical of the primitive and immature ENS during the early phase of hindgut development.
Persistent Identifierhttp://hdl.handle.net/10722/83739
ISSN
2015 Impact Factor: 1.733
2015 SCImago Journal Rankings: 0.802
ISI Accession Number ID

 

DC FieldValueLanguage
dc.contributor.authorTam, PKHen_HK
dc.contributor.authorGould, SJen_HK
dc.contributor.authorMartucciello, Gen_HK
dc.contributor.authorBiddolph, Sen_HK
dc.contributor.authorTakahashi, Men_HK
dc.contributor.authorJasonni, Ven_HK
dc.date.accessioned2010-09-06T08:44:38Z-
dc.date.available2010-09-06T08:44:38Z-
dc.date.issued1996en_HK
dc.identifier.citationJournal of Pediatric Surgery, 1996, v. 31 n. 4, p. 568-571en_HK
dc.identifier.issn0022-3468en_HK
dc.identifier.urihttp://hdl.handle.net/10722/83739-
dc.description.abstractA major gene for Hirschsprung's disease (HD) recently has been mapped in chromosome 10q11.2 and identified to be the RET proto-oncogene. Mutations of the RET gene have occurred in HD patients, and abnormalities of expression and function of Ret protein (a receptor tyrosine kinase, which is the product of the RET gene) have been found in their intestines. In vitro studies of the biological effects of HD mutations suggest a loss of function effect, which may be negative-dominant. However, the developmental role of the Ret protein in the organogenesis of the enteric nervous system (ENS) and its role in the pathogenesis of HD remain unclear. The authors present a study of the expression of Ret protein in the human ENS during fetal development. Fresh rectal tissues were obtained from nine fetuses (gestational age range, 12 to 22 weeks). Ret protein expression was studied immunohistochemically, using antibodies against the carboxy-terminal 20 amino acids (anti-Ret C) and the extracellular domain (anti-Ret R5). The tyrosine kinase activity of the fetal ENS was investigated with antiphosphotyrosine mouse monoclonal antibody against the phosphorylated tyrosine residues. Anti-Ret C immunostaining was observed in ganglion cells at all ages, but intense activity was significantly higher among the cells of the younger fetuses. Intense anti-Ret R5 immunostaining was present in the enteric ganglion cells of the 12-week-old fetus. The tyrosine kinase activity of ganglion cells increases progressively with advancing gestational age. The results of this study support the hypothesis that the Ret protein receptor might play a crucial role in the cellular and molecular processes involved in the development and maturation of the ENS, abnormalities of which could result in HD. High Ret protein expression and low tyrosine kinase activity have been reported to occur in small ganglia of the HD hypoganglionic segment. In the present study, these markers were typical of the primitive and immature ENS during the early phase of hindgut development.-
dc.languageengen_HK
dc.publisherWB Saunders Co. The Journal's web site is located at http://www.elsevier.com/locate/jpedsurgen_HK
dc.relation.ispartofJournal of Pediatric Surgeryen_HK
dc.subject.meshDrosophila Proteins-
dc.subject.meshEnteric Nervous System - embryology - pathology-
dc.subject.meshProto-Oncogene Proteins - genetics-
dc.subject.meshReceptor Protein-Tyrosine Kinases - genetics-
dc.subject.meshRectum - embryology - pathology-
dc.titleRet protein in the human fetal rectumen_HK
dc.typeArticleen_HK
dc.identifier.emailTam, PKH: paultam@hkucc.hku.hken_HK
dc.identifier.authorityTam, PKH=rp00060en_HK
dc.description.naturelink_to_subscribed_fulltext-
dc.identifier.doi10.1016/S0022-3468(96)90498-4-
dc.identifier.pmid8801315-
dc.identifier.hkuros23460en_HK
dc.identifier.volume31-
dc.identifier.issue4-
dc.identifier.spage568-
dc.identifier.epage571-
dc.identifier.isiWOS:A1996UE59000024-
dc.publisher.placeUnited States-

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