Extracellular elastic fibers are central to effective organ function, but the molecules that control the formation of these elastic fibers remains unclear. Two papers in January 10
Hiromi Yanagisawa and colleagues from University of Texas Southwestern Medical Centre, Dallas, observed that fibulin-5 knockout mice developed marked elastinopathy owing to the disorganization of elastic fibers, with resulting loose skin, vascular abnormalities and emphysematous lung (
In addition, a second paper by Tomoyuki Nakamura and colleagues from University of California, San Diego, confirms that fibulin-5 -/- mice exhibit a severely disorganized elastic fiber system throughout the body. These tissues contained fragmented elastin but showed no increase in elastase activity, indicating defective development of elastic fibers (
Both teams suggest...