作者
Morten A Karsdal, Tina Manon-Jensen, Federica Genovese, Jacob H Kristensen, Mette J Nielsen, Jannie Marie B Sand, Niels-Ulrik B Hansen, Anne-Christine Bay-Jensen, Cecilie L Bager, Aleksander Krag, Andy Blanchard, Henrik Krarup, Diana J Leeming, Detlef Schuppan
发表日期
2015/5/15
来源
American Journal of Physiology-Gastrointestinal and Liver Physiology
卷号
308
期号
10
页码范围
G807-G830
出版商
American Physiological Society
简介
Emerging evidence suggests that altered components and posttranslational modifications of proteins in the extracellular matrix (ECM) may both initiate and drive disease progression. The ECM is a complex grid consisting of multiple proteins, most of which play a vital role in containing the essential information needed for maintenance of a sophisticated structure anchoring the cells and sustaining normal function of tissues. Therefore, the matrix itself may be considered as a paracrine/endocrine entity, with more complex functions than previously appreciated. The aims of this review are to 1) explore key structural and functional components of the ECM as exemplified by monogenetic disorders leading to severe pathologies, 2) discuss selected pathological posttranslational modifications of ECM proteins resulting in altered functional (signaling) properties from the original structural proteins, and 3) discuss how these …
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MA Karsdal, T Manon-Jensen, F Genovese… - American Journal of Physiology-Gastrointestinal and …, 2015