Lack of beta1 integrins in enteric neural crest cells leads to a Hirschsprung-like phenotype.
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Lack of beta1 integrins in enteric neural crest cells leads to a Hirschsprung-like phenotype. / Breau, Marie A; Pietri, Thomas; Eder, Olivier; Blanche, Martine; Brakebusch, Cord; Fässler, Reinhardt; Thiery, Jean P; Dufour, Sylvie.
I: Development, Bind 133, Nr. 9, 2006, s. 1725-34.Publikation: Bidrag til tidsskrift › Tidsskriftartikel › Forskning › fagfællebedømt
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TY - JOUR
T1 - Lack of beta1 integrins in enteric neural crest cells leads to a Hirschsprung-like phenotype.
AU - Breau, Marie A
AU - Pietri, Thomas
AU - Eder, Olivier
AU - Blanche, Martine
AU - Brakebusch, Cord
AU - Fässler, Reinhardt
AU - Thiery, Jean P
AU - Dufour, Sylvie
N1 - Keywords: Animals; Antigens, CD29; Disease Models, Animal; Enteric Nervous System; Hirschsprung Disease; Immunohistochemistry; Integrases; Mice; Models, Genetic; Mutation; Neural Crest; Organ Culture Techniques; Phenotype; Viral Proteins
PY - 2006
Y1 - 2006
N2 - 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.
AB - 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.
U2 - 10.1242/dev.02346
DO - 10.1242/dev.02346
M3 - Journal article
C2 - 16571628
VL - 133
SP - 1725
EP - 1734
JO - Development
JF - Development
SN - 0950-1991
IS - 9
ER -
ID: 5141034