...
  • 主题
高级搜索  >
历史搜索 清空历史
首页> 外文期刊>Journal of Cellular Physiology >Fibrillin-rich microfibrils: Structural determinants of morphogenetic and homeostatic events.
【24h】

Fibrillin-rich microfibrils: Structural determinants of morphogenetic and homeostatic events.

机译：Fibrillin-rich微纤维:结构形态形成的决定因素和体内平衡事件。

获取原文
获取原文并翻译 | 示例
           
页面导航
  • 摘要
  • 著录项
  • 相似文献
  • 相关主题

摘要

Fibrillin-rich microfibrils are specialized extracellular matrix assemblies that endow connective tissues with mechanical stability and elastic properties, and that participate in the regulation of organ formation, growth and homeostasis. Their physiological importance is underscored by the complex spectrum of clinical manifestations associated with mutations of fibrillin-1 and fibrillin-2 in Marfan syndrome (MFS) and congenital contractural arachnodactyly, respectively. Early evidence suggested that fibrillin-1 mutations in MFS lead to loss of tissue integrity by perturbing microfibril assembly and function. Recent studies in genetically targeted mice have however revealed that fibrillin-1 and fibrillin-2 mutations perturb signaling events mediated by TGF-beta superfamily members. As such, these studies have established a new biological paradigm whereby fibrillin-rich microfibrils are structural networks that specify the local concentration and timely release of signaling molecules during morphogenesis and tissue remodeling. This review summarizes our current understanding of the role of fibrillin-rich microfibrils in development and disease, as well as exciting new applications in the clinical management of MFS and related connective tissue disorders.
机译：Fibrillin-rich微纤维专业细胞外基质赋予的程序集结缔组织和机械稳定性弹性性质,参与调节器官形成、生长和体内平衡。强调通过临床的复杂光谱表现与基因突变有关fibrillin-1和fibrillin-2马凡氏综合症(MFS)和先天性国民间细长指,分别。fibrillin-1 MFS的突变导致的损失由微扰微纤维组织完整性组装和功能。基因靶向老鼠但是透露fibrillin-1和fibrillin-2突变由及扰乱信号事件超家族成员。建立了一个新的生物范式,fibrillin-rich微纤维是结构性的网络指定地方浓度和及时释放的信号分子形态发生和组织重构。总结了我们当前对角色的理解fibrillin-rich的发展和微纤维疾病,以及令人兴奋的新的应用程序MFS和相关的临床管理结缔组织疾病。

著录项

相似文献

  • 外文文献
  • 中文文献
获取原文

客服邮箱：kefu@zhangqiaokeyan.com

京公网安备：11010802029741号 ICP备案号：京ICP备15016152号-6 六维联合信息科技 (北京) 有限公司©版权所有

  • 客服微信

  • 服务号