Imperial College London
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DrSalvatoreSantamaria

Faculty of Medicine, Department of Immunology and Inflammation

Honorary Lecturer
 
 
 
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Contact

 

s.santamaria

 
 
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Location

 

Commonwealth BuildingHammersmith Campus

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Summary

 

Publications

Citation

BibTex format

@article{Santamaria:2020:10.1098/rsob.200333,
author = {Santamaria, S and de, Groot R},
doi = {10.1098/rsob.200333},
journal = {Open Biology},
pages = {1--18},
title = {ADAMTS proteases in cardiovascular physiology and disease},
url = {http://dx.doi.org/10.1098/rsob.200333},
volume = {10},
year = {2020}
}
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RIS format (EndNote, RefMan)

TY  - JOUR
AB  - The a disintegrin-like and metalloproteinase with thrombospondin motif (ADAMTS) family comprises 19 proteases that regulate the structure and function of extracellular proteins in the extracellular matrix and blood. The best characterized cardiovascular role is that of ADAMTS-13 in blood. Moderately low ADAMTS-13 levels increase the risk of ischeamic stroke and very low levels (less than 10%) can cause thrombotic thrombocytopenic purpura (TTP). Recombinant ADAMTS-13 is currently in clinical trials for treatment of TTP. Recently, new cardiovascular roles for ADAMTS proteases have been discovered. Several ADAMTS family members are important in the development of blood vessels and the heart, especially the valves. A number of studies have also investigated the potential role of ADAMTS-1, -4 and -5 in cardiovascular disease. They cleave proteoglycans such as versican, which represent major structural components of the arteries. ADAMTS-7 and -8 are attracting considerable interest owing to their implication in atherosclerosis and pulmonary arterial hypertension, respectively. Mutations in the ADAMTS19 gene cause progressive heart valve disease and missense variants in ADAMTS6 are associated with cardiac conduction. In this review, we discuss in detail the evidence for these and other cardiovascular roles of ADAMTS family members, their proteolytic substrates and the potential molecular mechanisms involved.
AU  - Santamaria,S
AU  - de,Groot R
DO  - 10.1098/rsob.200333
EP  - 18
PY  - 2020///
SN  - 2046-2441
SP  - 1
TI  - ADAMTS proteases in cardiovascular physiology and disease
T2  - Open Biology
UR  - http://dx.doi.org/10.1098/rsob.200333
UR  - http://gateway.webofknowledge.com/gateway/Gateway.cgi?GWVersion=2&SrcApp=PARTNER_APP&SrcAuth=LinksAMR&KeyUT=WOS:000603076400001&DestLinkType=FullRecord&DestApp=ALL_WOS&UsrCustomerID=1ba7043ffcc86c417c072aa74d649202
UR  - https://royalsocietypublishing.org/doi/10.1098/rsob.200333
UR  - http://hdl.handle.net/10044/1/91435
VL  - 10
ER  -
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