Literature DB >> 15234214

A genetic mechanism for cecal atresia: the role of the Fgf10 signaling pathway.

T J Fairbanks1, R C Kanard, S P De Langhe, F G Sala, P M Del Moral, D Warburton, K D Anderson, S Bellusci, R C Burns.   

Abstract

BACKGROUND: Intestinal atresia represents a significant surgically correctable cause of intestinal obstruction in neonates. Intestinal development proceeds as a tube-like structure with differentiation along its axis. As the intestine differentiates, the cecum develops at the transition from small to large intestine. Fgf10 is known to serve a key role in budding morphogenesis; however, little is known about its role in the development of this transitional structure. Here we evaluate the effect of Fgf10/Fgfr2b invalidation on the developing cecum.
MATERIALS AND METHODS: Wild-type C57Bl/6, Fgf10(-/-), and Fgfr2b(-/-) embryos harvested from timed pregnant mothers were analyzed for cecal phenotype, Fgf10 expression, and differentiation of smooth muscle actin.
RESULTS: Wt cecal development is first evident at E11.5. FGF10 is discreetly expressed in the area of the developing cecum at early stages of development. One hundred percent of Fgf10(-/-) and Fgfr2b(-/-) mutant embryos demonstrate cecal atresia with absence of epithelial and muscular layers. The development of neighboring anatomical structures such as the ileocecal valve is not affected by Fgf10/Fgfr2b invalidation.
CONCLUSIONS: FGF10 expression is localized to the cecum early in the normal development of the cecum. Fgf10(-/-) and Fgfr2b(-/-) mutant embryos demonstrate cecal atresia with complete penetrance. Epithelial and muscular layers of the cecum are not present in the atretic cecum. The Fgf10(-/-) and Fgfr2b(-/-) mutants represent a genetically reproducible animal model of autosomal recessive intestinal atresia.

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Year:  2004        PMID: 15234214     DOI: 10.1016/j.jss.2003.12.017

Source DB:  PubMed          Journal:  J Surg Res        ISSN: 0022-4804            Impact factor:   2.192


  11 in total

1.  Fibroblast growth factor 10 alters the balance between goblet and Paneth cells in the adult mouse small intestine.

Authors:  Denise Al Alam; Soula Danopoulos; Kathy Schall; Frederic G Sala; Dana Almohazey; G Esteban Fernandez; Senta Georgia; Mark R Frey; Henri R Ford; Tracy Grikscheit; Saverio Bellusci
Journal:  Am J Physiol Gastrointest Liver Physiol       Date:  2015-02-26       Impact factor: 4.052

2.  FGF9-Pitx2-FGF10 signaling controls cecal formation in mice.

Authors:  Denise Al Alam; Frederic G Sala; Sheryl Baptista; Rosanna Galzote; Soula Danopoulos; Caterina Tiozzo; Philip Gage; Tracy Grikscheit; David Warburton; Mark R Frey; Saverio Bellusci
Journal:  Dev Biol       Date:  2012-07-20       Impact factor: 3.582

3.  Lack of discreet colocalization of epithelial apoptosis to the atretic precursor in the colon of the Fibroblast growth factor receptor 2IIIb mouse and staining consistent with cellular movement suggest a revised model of atresia formation.

Authors:  Anna Kowalkowski; Krzysztof M Zaremba; Andrew P Rogers; Olivia R Hoffman; Anne E Turco; Peter F Nichol
Journal:  Dev Dyn       Date:  2020-03-10       Impact factor: 3.780

Review 4.  Humans, mice, and mechanisms of intestinal atresias: a window into understanding early intestinal development.

Authors:  Peter F Nichol; Amy Reeder; Robert Botham
Journal:  J Gastrointest Surg       Date:  2010-11-30       Impact factor: 3.452

5.  Fibroblast growth factor 10 represses premature cell differentiation during establishment of the intestinal progenitor niche.

Authors:  Pia Nyeng; Maureen Ann Bjerke; Gitte Anker Norgaard; Xiaoling Qu; Sune Kobberup; Jan Jensen
Journal:  Dev Biol       Date:  2010-09-27       Impact factor: 3.582

Review 6.  Vertebrate intestinal endoderm development.

Authors:  Jason R Spence; Ryan Lauf; Noah F Shroyer
Journal:  Dev Dyn       Date:  2011-01-18       Impact factor: 3.780

7.  Duodenal atresia associated with "apple peel" small bowel without deletion of fibroblast growth factor-10 or fibroblast growth factor receptor 2IIIb: report of a case.

Authors:  Yukihiro Tatekawa; Hiromichi Kanehiro; Yoshiyuki Nakajima
Journal:  Surg Today       Date:  2007-04-30       Impact factor: 2.549

8.  Characterization of a novel fibroblast growth factor 10 (Fgf10) knock-in mouse line to target mesenchymal progenitors during embryonic development.

Authors:  Elie El Agha; Denise Al Alam; Gianni Carraro; Breanne MacKenzie; Kerstin Goth; Stijn P De Langhe; Robert Voswinckel; Mohammad K Hajihosseini; Virender K Rehan; Saverio Bellusci
Journal:  PLoS One       Date:  2012-06-13       Impact factor: 3.240

9.  Influenza Virus Infects Epithelial Stem/Progenitor Cells of the Distal Lung: Impact on Fgfr2b-Driven Epithelial Repair.

Authors:  Jennifer Quantius; Carole Schmoldt; Ana I Vazquez-Armendariz; Christin Becker; Elie El Agha; Jochen Wilhelm; Rory E Morty; István Vadász; Konstantin Mayer; Stefan Gattenloehner; Ludger Fink; Mikhail Matrosovich; Xiaokun Li; Werner Seeger; Juergen Lohmeyer; Saverio Bellusci; Susanne Herold
Journal:  PLoS Pathog       Date:  2016-06-20       Impact factor: 6.823

10.  Fibroblast growth factor 10-fibroblast growth factor receptor 2b mediated signaling is not required for adult glandular stomach homeostasis.

Authors:  Allison L Speer; Denise Al Alam; Frederic G Sala; Henri R Ford; Saverio Bellusci; Tracy C Grikscheit
Journal:  PLoS One       Date:  2012-11-01       Impact factor: 3.240

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