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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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| Veröffentlicht in: | Cell Mol Life Sci |
|---|---|
| Hauptverfasser: | , |
| Format: | Artigo |
| Sprache: | Inglês |
| Veröffentlicht: |
SP Birkhäuser Verlag Basel
2011
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| Schlagworte: | |
| Online Zugang: | 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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