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dc.contributor.authorAtaca, D
dc.contributor.authorCaikovski, M
dc.contributor.authorPiersigilli, A
dc.contributor.authorMoulin, A
dc.contributor.authorBenarafa, C
dc.contributor.authorEarp, SE
dc.contributor.authorGuri, Y
dc.contributor.authorKostic, C
dc.contributor.authorArsenijevic, Y
dc.contributor.authorSoininen, R
dc.contributor.authorApte, SS
dc.contributor.authorBrisken, C
dc.date.accessioned2021-11-19T13:24:05Z
dc.date.available2021-11-19T13:24:05Z
dc.date.issued2016-11-15
dc.identifier.citationBiology open, 2016, 5 (11), pp. 1585 - 1594
dc.identifier.issn2046-6390
dc.identifier.urihttps://repository.icr.ac.uk/handle/internal/4887
dc.identifier.eissn2046-6390
dc.identifier.doi10.1242/bio.019711
dc.description.abstractThe 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.
dc.formatElectronic
dc.format.extent1585 - 1594
dc.languageeng
dc.language.isoeng
dc.rights.urihttps://creativecommons.org/licenses/by/4.0
dc.titleAdamts18 deletion results in distinct developmental defects and provides a model for congenital disorders of lens, lung, and female reproductive tract development.
dc.typeJournal Article
rioxxterms.versionVoR
rioxxterms.versionofrecord10.1242/bio.019711
rioxxterms.licenseref.urihttps://creativecommons.org/licenses/by/4.0
rioxxterms.licenseref.startdate2016-11-15
rioxxterms.typeJournal Article/Review
dc.relation.isPartOfBiology open
pubs.issue11
pubs.notesNot known
pubs.organisational-group/ICR
pubs.organisational-group/ICR/Primary Group
pubs.organisational-group/ICR/Primary Group/ICR Divisions
pubs.organisational-group/ICR/Primary Group/ICR Divisions/Breast Cancer Research
pubs.organisational-group/ICR/Primary Group/ICR Divisions/Breast Cancer Research/Endocrine control mechanisms
pubs.publication-statusPublished
pubs.volume5
pubs.embargo.termsNot known
icr.researchteamEndocrine control mechanisms
dc.contributor.icrauthorBrisken, Cathrinen_US


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