Adamts18 deletion results in distinct developmental defects and provides a model for congenital disorders of lens, lung, and female reproductive tract development.

Détails

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Etat: Serval
Version: Final published version
ID Serval
serval:BIB_BD206DF55CF5
Type
Article: article d'un périodique ou d'un magazine.
Collection
Publications
Titre
Adamts18 deletion results in distinct developmental defects and provides a model for congenital disorders of lens, lung, and female reproductive tract development.
Périodique
Biology open
Auteur(s)
Ataca D., Caikovski M., Piersigilli A., Moulin A., Benarafa C., Earp S.E., Guri Y., Kostic C., Arsenivic Y., Soininen R., Apte S.S., Brisken C.
ISSN-L
2046-6390
Statut éditorial
Publié
Date de publication
15/11/2016
Peer-reviewed
Oui
Volume
5
Numéro
11
Pages
1585-1594
Langue
anglais
Notes
Publication types: Journal Article
Publication Status: epublish
Résumé
The ADAMTS family comprises 19 secreted metalloproteinases that cleave extracellular matrix components and have diverse functions in numerous disease and physiological contexts. A number of them remain 'orphan' proteases and among them is ADAMTS18, which has been implicated in developmental eye disorders, platelet function and various malignancies. To assess in vivo function of ADAMTS18, we generated a mouse strain with inactivated Adamts18 alleles. In the C57Bl6/Ola background, Adamts18-deficient mice are born in a normal Mendelian ratio, and are viable but show a transient growth delay. Histological examination revealed a 100% penetrant eye defect resulting from leakage of lens material through the lens capsule occurring at embryonic day (E)13.5, when the lens grows rapidly. Adamts18-deficient lungs showed altered bronchiolar branching. Fifty percent of mutant females are infertile because of vaginal obstruction due to either a dorsoventral vaginal septum or imperforate vagina. The incidence of ovarian rete is increased in the mutant mouse strain. Thus, Adamts18 is essential in the development of distinct tissues and the new mouse strain is likely to be useful for investigating ADAMTS18 function in human disease, particularly in the contexts of infertility and carcinogenesis.

Pubmed
Web of science
Open Access
Oui
Création de la notice
15/12/2016 15:09
Dernière modification de la notice
09/05/2019 0:32
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