Adamts10 inactivation in mice leads to persistence of ocular microfibrils subsequent to reduced fibrillin-2 cleavage

Mutations in the secreted metalloproteinase ADAMTS10 cause recessive Weill-Marchesani syndrome (WMS), comprising ectopia lentis, short stature, brachydactyly, thick skin and cardiac valve anomalies. Dominant WMS caused by FBN1 mutations is clinically similar and affects fibrillin-1 microfibrils, whi...

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Publicat a:Matrix Biol
Autors principals: Wang, Lauren W., Kutz, Wendy E., Mead, Timothy J., Beene, Lauren C., Singh, Shweta, Jenkins, Michael W., Reinhardt, Dieter P., Apte, Suneel S.
Format: Artigo
Idioma:Inglês
Publicat: 2018
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Article
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC8209899/
https://ncbi.nlm.nih.gov/pubmed/30201140
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.matbio.2018.09.004
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