当前位置: X-MOL 学术CSH Perspect. Biol. › 论文详情
Our official English website, www.x-mol.net, welcomes your feedback! (Note: you will need to create a separate account there.)
TGF-β Family Signaling in Connective Tissue and Skeletal Diseases.
Cold Spring Harbor Perspectives in Biology ( IF 7.2 ) Pub Date : 2017-03-02 , DOI: 10.1101/cshperspect.a022269
Elena Gallo MacFarlane 1 , Julia Haupt 2, 3 , Harry C Dietz 1, 4 , Eileen M Shore 2, 3, 5
Affiliation  

The transforming growth factor β (TGF-β) family of signaling molecules, which includes TGF-βs, activins, inhibins, and numerous bone morphogenetic proteins (BMPs) and growth and differentiation factors (GDFs), has important functions in all cells and tissues, including soft connective tissues and the skeleton. Specific TGF-β family members play different roles in these tissues, and their activities are often balanced with those of other TGF-β family members and by interactions with other signaling pathways. Perturbations in TGF-β family pathways are associated with numerous human diseases with prominent involvement of the skeletal and cardiovascular systems. This review focuses on the role of this family of signaling molecules in the pathologies of connective tissues that manifest in rare genetic syndromes (e.g., syndromic presentations of thoracic aortic aneurysm), as well as in more common disorders (e.g., osteoarthritis and osteoporosis). Many of these diseases are caused by or result in pathological alterations of the complex relationship between the TGF-β family of signaling mediators and the extracellular matrix in connective tissues.

中文翻译:

结缔组织和骨骼疾病中的TGF-β家族信号传导。

信号转导分子的转化生长因子β(TGF-β)家族,包括TGF-β,激活素,抑制剂和许多骨形态发生蛋白(BMP)和生长分化因子(GDF),在所有细胞和组织中都具有重要功能包括软结缔组织和骨骼。特定的TGF-β家族成员在这些组织中起着不同的作用,并且它们的活性通常与其他TGF-β家族成员的活性以及与其他信号通路的相互作用相平衡。TGF-β家族通路的扰动与许多人类疾病有关,骨骼和心血管系统也参与其中。这篇综述着重于这一信号分子家族在罕见遗传综合症(例如,胸主动脉瘤的症状表现,以及更常见的疾病(例如骨关节炎和骨质疏松症)。这些疾病中的许多是由TGF-β家族信号传导介质与结缔组织中细胞外基质之间复杂关系引起或导致的病理改变。
更新日期:2019-11-01
down
wechat
bug