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

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 diso...

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Main Authors:	Dalya Ataca, Marian Caikovski, Alessandra Piersigilli, Alexandre Moulin, Charaf Benarafa, Sarah E. Earp, Yakir Guri, Corinne Kostic, Yvan Arsenivic, Raija Soininen, Suneel S. Apte, Cathrin Brisken
Format:	Article
Language:	English
Published:	The Company of Biologists 2016-11-01
Series:	Biology Open
Subjects:	Adamts18 Metalloproteinase Lens capsule Lung development Vaginal septum Rete ovary
Online Access:	http://bio.biologists.org/content/5/11/1585

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spelling	doaj-864fa76dacce443db8a7cb36e25536c82021-06-02T17:52:29ZengThe Company of BiologistsBiology Open2046-63902016-11-015111585159410.1242/bio.019711019711Adamts18 deletion results in distinct developmental defects and provides a model for congenital disorders of lens, lung, and female reproductive tract developmentDalya Ataca0Marian Caikovski1Alessandra Piersigilli2Alexandre Moulin3Charaf Benarafa4Sarah E. Earp5Yakir Guri6Corinne Kostic7Yvan Arsenivic8Raija Soininen9Suneel S. Apte10Cathrin Brisken11 Ecole Polytechnique Fédérale de Lausanne, ISREC, NCCR Molecular Oncology, Station 19, Lausanne CH-1015, Switzerland Ecole Polytechnique Fédérale de Lausanne, ISREC, NCCR Molecular Oncology, Station 19, Lausanne CH-1015, Switzerland Ecole Polytechnique Fédérale de Lausanne, ISREC, NCCR Molecular Oncology, Station 19, Lausanne CH-1015, Switzerland Jules-Gonin Eye Hospital, University of Lausanne, Avenue de France 15, Lausanne CH-1004, Switzerland Theodor Kocher Institute, University of Bern, Freiestrasse 1, Bern CH-3012, Switzerland Biomedical Engineering-ND20, Cleveland Clinic Lerner Research Institute, 9500 Euclid Ave., Cleveland, OH 44195, USA Ecole Polytechnique Fédérale de Lausanne, ISREC, NCCR Molecular Oncology, Station 19, Lausanne CH-1015, Switzerland Jules-Gonin Eye Hospital, University of Lausanne, Avenue de France 15, Lausanne CH-1004, Switzerland Jules-Gonin Eye Hospital, University of Lausanne, Avenue de France 15, Lausanne CH-1004, Switzerland Department of Pathology, Biocenter Oulu, University of Oulu, Oulu FIN-90014, Finland Biomedical Engineering-ND20, Cleveland Clinic Lerner Research Institute, 9500 Euclid Ave., Cleveland, OH 44195, USA Ecole Polytechnique Fédérale de Lausanne, ISREC, NCCR Molecular Oncology, Station 19, Lausanne CH-1015, Switzerland 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.http://bio.biologists.org/content/5/11/1585Adamts18MetalloproteinaseLens capsuleLung developmentVaginal septumRete ovary
collection	DOAJ
language	English
format	Article
sources	DOAJ
author	Dalya Ataca Marian Caikovski Alessandra Piersigilli Alexandre Moulin Charaf Benarafa Sarah E. Earp Yakir Guri Corinne Kostic Yvan Arsenivic Raija Soininen Suneel S. Apte Cathrin Brisken
spellingShingle	Dalya Ataca Marian Caikovski Alessandra Piersigilli Alexandre Moulin Charaf Benarafa Sarah E. Earp Yakir Guri Corinne Kostic Yvan Arsenivic Raija Soininen Suneel S. Apte Cathrin Brisken Adamts18 deletion results in distinct developmental defects and provides a model for congenital disorders of lens, lung, and female reproductive tract development Biology Open Adamts18 Metalloproteinase Lens capsule Lung development Vaginal septum Rete ovary
author_facet	Dalya Ataca Marian Caikovski Alessandra Piersigilli Alexandre Moulin Charaf Benarafa Sarah E. Earp Yakir Guri Corinne Kostic Yvan Arsenivic Raija Soininen Suneel S. Apte Cathrin Brisken
author_sort	Dalya Ataca
title	Adamts18 deletion results in distinct developmental defects and provides a model for congenital disorders of lens, lung, and female reproductive tract development
title_short	Adamts18 deletion results in distinct developmental defects and provides a model for congenital disorders of lens, lung, and female reproductive tract development
title_full	Adamts18 deletion results in distinct developmental defects and provides a model for congenital disorders of lens, lung, and female reproductive tract development
title_fullStr	Adamts18 deletion results in distinct developmental defects and provides a model for congenital disorders of lens, lung, and female reproductive tract development
title_full_unstemmed	Adamts18 deletion results in distinct developmental defects and provides a model for congenital disorders of lens, lung, and female reproductive tract development
title_sort	adamts18 deletion results in distinct developmental defects and provides a model for congenital disorders of lens, lung, and female reproductive tract development
publisher	The Company of Biologists
series	Biology Open
issn	2046-6390
publishDate	2016-11-01
description	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.
topic	Adamts18 Metalloproteinase Lens capsule Lung development Vaginal septum Rete ovary
url	http://bio.biologists.org/content/5/11/1585
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Adamts18 deletion results in distinct developmental defects and provides a model for congenital disorders of lens, lung, and female reproductive tract development

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