Mice that lack this matrix protein, called fibulin-5, have loose, wrinkled skin, vascular abnormalities, and emphysema, all of which are thought to be due to their disorganized and fragmented elastic fibers.
Elastic fibers are composed of an inner core of cross-linked elastin surrounded by a microfibril envelope. Hirai et al. now show that fibulin-5 generates elastic fibers by promoting aggregation of the elastin precursor, called tropoelastin, its deposition onto microfibrils, and its cross-linking into mature elastin.
A naturally occurring truncated version of fibulin-5 that did not associate with microfibrils was unable...
The Rockefeller University Press
2007
The Rockefeller University Press
2007
You do not currently have access to this content.