Extracellular microfibrils in development and disease.
Fibrillins are the structural components of extracellular microfibrils that impart physical properties to tissues, alone or together with elastin as elastic fibers. Genetic studies in mice have revealed that fibrillin-rich microfibrils are also involved in regulating developmental programs and homeostatic processes through the modulation of TGF-beta/BMP signaling events. A new paradigm has thus emerged whereby the spatiotemporal organization of microfibrils dictates both the cellular activities and physical properties of connective tissues. These observations have paved the way to novel therapeutic approaches aimed at counteracting the life-threatening complications in human conditions caused by dysfunctions of fibrillin-rich microfibrils.
Ramirez
F
F
Department of Pediatrics, Child Health Institute of New Jersey, UMDNJ-Robert Wood Johnson Medical School, 89 French Street, New Brunswick, NJ 08901, USA. ramirefr@umdnj.edu
Sakai
L Y
LY
Rifkin
D B
DB
Dietz
H C
HC
D000818:Animals; D002318:Cardiovascular Diseases / Q000378:metabolism*; D005109:Extracellular Matrix / Q000737:chemistry / Q000378:metabolism*; D000071837:Fibrillins; D006801:Humans; D020894:Microfibrils / Q000737:chemistry / Q000378:metabolism*; D008840:Microfilament Proteins / Q000737:chemistry / Q000378:metabolism; D009024:Morphogenesis
D000071837:Fibrillins; D008840:Microfilament Proteins