Article
Early fibrillin-1 assembly monitored through a modifiable recombinant cell approach.
Biomacromolecules - 14 Apr 2014
Hubmacher Dirk, Bergeron Eric, Fagotto-Kaufmann Christine, Sakai Lynn Y, Reinhardt Dieter P
Abstract excerpt
Fibrillin proteins constitute the backbone of extra-cellular 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 microfibril assembly in cell culture models. Full length recombinant fibrillin-1 was expressed by HEK 293 cells, which...
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