发育与疾病中的细胞外微原纤维
Extracellular microfibrils in development and disease.
作者信息
Ramirez F, Sakai L Y, Rifkin D B, Dietz H C
机构信息
Department of Pediatrics, Child Health Institute of New Jersey, UMDNJ-Robert Wood Johnson Medical School, 89 French Street, New Brunswick, NJ 08901, USA.
出版信息
Cell Mol Life Sci. 2007 Sep;64(18):2437-46. doi: 10.1007/s00018-007-7166-z.
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.
原纤维蛋白是细胞外微原纤维的结构成分，它们单独或与弹性蛋白一起作为弹性纤维赋予组织物理特性。对小鼠的遗传学研究表明，富含原纤维蛋白的微原纤维还通过调节转化生长因子-β/骨形态发生蛋白（TGF-β/BMP）信号事件参与调控发育程序和稳态过程。因此，一个新的范例出现了，即微原纤维的时空组织决定了结缔组织的细胞活动和物理特性。这些观察结果为旨在对抗由富含原纤维蛋白的微原纤维功能障碍引起的危及生命并发症的新型治疗方法铺平了道路。
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