Lack of beta1 integrins in enteric neural crest cells leads to a Hirschsprung-like phenotype
| Autor: | Cord Brakebusch, Martine Blanche, Marie A. Breau, Sylvie Dufour, Reinhardt Fässler, Jean P. Thiery, Thomas Pietri, Olivier Eder |
|---|---|
| Přispěvatelé: | Compartimentation et dynamique cellulaires (CDC), Centre National de la Recherche Scientifique (CNRS)-Institut Curie [Paris]-Université Pierre et Marie Curie - Paris 6 (UPMC), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut Curie [Paris]-Centre National de la Recherche Scientifique (CNRS) |
| Jazyk: | angličtina |
| Rok vydání: | 2006 |
| Předmět: |
[SDV]Life Sciences [q-bio]
Integrin Enteric Nervous System Extracellular matrix Mice Viral Proteins 03 medical and health sciences Organ Culture Techniques 0302 clinical medicine Conditional gene knockout medicine Animals Hirschsprung Disease Molecular Biology Hirschsprung's disease ComputingMilieux_MISCELLANEOUS 030304 developmental biology 0303 health sciences Integrases Models Genetic biology Integrin beta1 Neural tube Neural crest medicine.disease Immunohistochemistry Small intestine Cell biology Disease Models Animal Phenotype medicine.anatomical_structure Neural Crest Mutation Immunology biology.protein Enteric nervous system 030217 neurology & neurosurgery Developmental Biology |
| Zdroj: | Development (Cambridge, England) Development (Cambridge, England), Company of Biologists, 2006, 133 (9), pp.1725-1734. ⟨10.1242/dev.02346⟩ Development (Cambridge, England), 2006, 133 (9), pp.1725-1734. ⟨10.1242/dev.02346⟩ |
| ISSN: | 1477-9129 0950-1991 |
| DOI: | 10.1242/dev.02346⟩ |
| Popis: | The enteric nervous system arises mainly from vagal and sacral neural crest cells that colonise the gut between 9.5 and 14 days of development in mice. Using the Cre-LoxP system, we removed beta1 integrins in the neural crest cells when they emerge from the neural tube. beta1-null enteric neural crest cells fail to colonise the gut completely, leading to an aganglionosis of the descending colon, which resembles the human Hirschsprung's disease. Moreover, beta1-null enteric neural crest cells form abnormal aggregates in the gut wall, leading to a severe alteration of the ganglia network organisation. Organotypic cultures of gut explants reveal that beta1-null enteric neural crest cells show impaired adhesion on extracellular matrix and enhanced intercellular adhesion properties. They display migration defects in collagen gels and gut tissue environments. We also provide evidence that beta1 integrins are required for the villi innervation in the small intestine. Our findings highlight the crucial roles played by beta1 integrins at various steps of enteric nervous system development. |
| Databáze: | OpenAIRE |
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