000221414 001__ 221414
000221414 005__ 20190317000533.0
000221414 0247_ $$2doi$$a10.1242/bio.019711
000221414 022__ $$a2046-6390
000221414 02470 $$2ISI$$a000388302100004
000221414 037__ $$aARTICLE
000221414 245__ $$aAdamts18 deletion results in distinct developmental defects and provides a model for congenital disorders of lens, lung, and female reproductive tract development
000221414 269__ $$a2016
000221414 260__ $$bCompany Of Biologists Ltd$$c2016$$aCambridge
000221414 300__ $$a10
000221414 336__ $$aJournal Articles
000221414 520__ $$aThe 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, among them 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 the 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 mice. 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.
000221414 6531_ $$aAdamts18
000221414 6531_ $$aMetalloproteinase
000221414 6531_ $$aLens capsule
000221414 6531_ $$aLung development
000221414 6531_ $$aVaginal septum
000221414 6531_ $$aRete ovary
000221414 700__ $$0247548$$g236535$$aAtaca, Dalya
000221414 700__ $$0240288$$g169115$$aCaikovski, Marian
000221414 700__ $$aPiersigilli, Alessandra
000221414 700__ $$aMoulin, Alexandre
000221414 700__ $$aBenarafa, Charaf
000221414 700__ $$aEarp, Sarah E
000221414 700__ $$0245918$$g220199$$aGuri, Yakir
000221414 700__ $$aKostic, Corinne
000221414 700__ $$aArsenivic, Yvan
000221414 700__ $$aSoininen, Raija
000221414 700__ $$aApte, Suneel S
000221414 700__ $$aBrisken, Cathrin$$g169115$$0240288
000221414 773__ $$j5$$tBiology open$$q1585-1594
000221414 8564_ $$uhttps://infoscience.epfl.ch/record/221414/files/bio.019711.full.pdf$$zPublisher's version$$s2481878$$yPublisher's version
000221414 909C0 $$xU11257$$0252103$$pUPBRI
000221414 909CO $$qGLOBAL_SET$$pSV$$ooai:infoscience.tind.io:221414$$particle
000221414 917Z8 $$x182396
000221414 917Z8 $$x148230
000221414 937__ $$aEPFL-ARTICLE-221414
000221414 973__ $$rREVIEWED$$sPUBLISHED$$aEPFL
000221414 980__ $$aARTICLE