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Genetic and functional linkage between ADAMTS superfamily proteins and fibrillin-1: a novel mechanism influencing microfibril assembly and function
Tissue microfibrils contain fibrillin-1 as a major constituent. Microfibrils regulate bioavailability of TGFβ superfamily growth factors and are structurally crucial in the ocular zonule. FBN1 mutations typically cause the Marfan syndrome, an autosomal dominant disorder manifesting with skeletal ove...
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| 出版年: | Cell Mol Life Sci |
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| 主要な著者: | , |
| フォーマット: | Artigo |
| 言語: | Inglês |
| 出版事項: |
SP Birkhäuser Verlag Basel
2011
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| 主題: | |
| オンライン･アクセス: | https://ncbi.nlm.nih.gov/pmc/articles/PMC4729447/ https://ncbi.nlm.nih.gov/pubmed/21858451 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1007/s00018-011-0780-9 |
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