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Extracellular microfibrils in development and disease |
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| Authors: | F Ramirez L Y Sakai D B Rifkin H C Dietz |
| Institution: | (1) Department of Pediatrics, Child Health Institute of New Jersey, UMDNJ-Robert Wood Johnson Medical School, 89 French Street, New Brunswick, NJ 08901, USA;(2) Research Department, Shriners Hospital for Children, Portland, OR 97201, USA;(3) Department of Cell Biology, New York University School of Medicine, New York, NY 10016, USA;(4) Departments of Pediatrics, Medicine and Molecular Biology and Genetics, and Howard Hughes Medical Institute, John Hopkins University School of Medicine, Baltimore, MD 21205, USA |
| Abstract: | 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-β/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. Received 2 April 2007; received after revision 23 May 2007; accepted 24 May 2007 |
| Keywords: | Aortic aneurysm development and morphogenesis emphysema fibrillin extracellular matrix Marfan syndrome myopathy TGF-β /BMP signaling molecules |
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