Fibrillin microfibrils and elastic fibre proteins: Functional interactions and extracellular regulation of growth factors.
Fibrillin microfibrils and elastic fibre proteins: Functional interactions and extracellular regulation of growth factors.
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DOI：
10.1016/j.semcdb.2018.07.016
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发表时间：
2019-05
影响因子：
7.3
通讯作者：
Baldock C
中科院分区：
文献类型：
--
作者：
Thomson J;Singh M;Eckersley A;Cain SA;Sherratt MJ;Baldock C
原纤蛋白微纤维是赋予结缔组织长距离弹性的可伸展聚合物，并且在弹性和非弹性组织中具有广泛的分布。它们在弹性纤维形成期间充当弹性蛋白沉积的模板，并且对于维持组织（例如血管、肺、皮肤和眼韧带）的完整性是必不可少的。在血管老化、慢性阻塞性肺疾病、皮肤老化和紫外线引起的皮肤损伤以及与年龄相关的视力退化的组织中观察到维生素B1的减少。马凡氏综合征是一种遗传性疾病，其特征是长骨过度生长和其他骨骼异常，伴有心血管和眼睛缺陷。然而，突变的蛋白质和原纤维蛋白结合蛋白也可以导致身材矮小的病理。所有这些疾病都与生长因子信号传导失调有关，生长因子信号传导失调形成了BERGIN的主要功能作用。
Fibrillin microfibrils are extensible polymers that endow connective tissues with long-range elasticity and have widespread distributions in both elastic and non-elastic tissues. They act as a template for elastin deposition during elastic fibre formation and are essential for maintaining the integrity of tissues such as blood vessels, lung, skin and ocular ligaments. A reduction in fibrillin is seen in tissues in vascular ageing, chronic obstructive pulmonary disease, skin ageing and UV induced skin damage, and age-related vision deterioration. Most mutations in fibrillin cause Marfan syndrome, a genetic disease characterised by overgrowth of the long bones and other skeletal abnormalities with cardiovascular and eye defects. However, mutations in fibrillin and fibrillin-binding proteins can also cause short-stature pathologies. All of these diseases have been linked to dysregulated growth factor signalling which forms a major functional role for fibrillin.
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影响因子：
4.1
作者：
Chandra, Aman;Jones, Megan;Charteris, David G.
通讯作者：
Charteris, David G.
DOI：
10.1073/pnas.0601609103
发表时间：
2006-08-08
影响因子：
11.1
作者：
Baldock, Clair;Siegler, Veronique;Wess, Tim J.
通讯作者：
Wess, Tim J.
影响因子：
4.3
作者：
Bultmann-Mellin I;Conradi A;Maul AC;Dinger K;Wempe F;Wohl AP;Imhof T;Wunderlich FT;Bunck AC;Nakamura T;Koli K;Bloch W;Ghanem A;Heinz A;von Melchner H;Sengle G;Sterner-Kock A
通讯作者：
Sterner-Kock A
影响因子：
2.9
作者：
Clarke, AW;Wise, SG;Weiss, AS
通讯作者：
Weiss, AS
fibulin-4 和-5 对弹性纤维形成的差异调节。
影响因子：
4.8
作者：
Choudhury, Rawshan;McGovern, Amanda;Ridley, Caroline;Cain, Stuart A.;Baldwin, Andrew;Wang, Ming-Chuan;Guo, Chun;Mironov, Aleksandr, Jr.;Drymoussi, Zoe;Trump, Dorothy;Shuttleworth, Adrian;Baldock, Clair;Kielty, Cay M.
通讯作者：
Kielty, Cay M.