Fibrillin microfibrils in bone physiology.
Fibrillin microfibrils in bone physiology.
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DOI：
10.1016/j.matbio.2015.09.004
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发表时间：
2016-05
期刊：
影响因子：
--
通讯作者：
Ramirez F
中科院分区：
文献类型：
--
作者：
Smaldone S;Ramirez F
The severe skeletal abnormalities associated with Marfan syndrome (MFS) and congenital contractural arachnodactyly (CCA) underscore the notion that fibrillin assemblies (microfibrils and elastic fibers) play a critical role in bone formation and function in spite of representing a low abundance component of skeletal matrices. Studies of MFS and CCA mice have correlated the skeletal phenotypes of these mutant animals with distinct pathophysiological mechanisms that reflect the contextual contribution of fibrillin-1 and -2 scaffolds to TGFβ and BMP signaling during bone patterning, growth and metabolism. Illustrative examples include the unique role of fibrillin-2 in regulating BMP-dependent limb patterning and the distinct impact of the two fibrillin proteins on the commitment and differentiation of marrow mesenchymal stem cells. Collectively, these findings have important implication for our understanding of the pathophysiological mechanisms that drive age- and injury-related processes of bone degeneration.
DOI：
10.1084/jem.2121insight2
发表时间：
2015-01-12
期刊：
The Journal of experimental medicine
影响因子：
--
作者：
Karsenty G
通讯作者：
Karsenty G