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Early fibrillin-1 assembly monitored through a modifiable recombinant cell approach

Fibrillin proteins constitute the backbone of extracellular macromolecular microfibrils. Mutations in fibrillins cause heritable connective tissue disorders, including Marfan syndrome, dominant Weill-Marchesani syndrome, and stiff skin syndrome. Fibronectin provides a critical scaffold for microfibr...

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Détails bibliographiques
Publié dans:Biomacromolecules
Auteurs principaux: Hubmacher, Dirk, Bergeron, Eric, Fagotto-Kaufmann, Christine, Sakai, Lynn Y., Reinhardt, Dieter P.
Format: Artigo
Langue:Inglês
Publié: 2014
Sujets:
Accès en ligne:https://ncbi.nlm.nih.gov/pmc/articles/PMC4961472/
https://ncbi.nlm.nih.gov/pubmed/24559401
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1021/bm5000696
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