ADAMTS proteases in cardiovascular physiology and disease.

Published on Dec 23, 2020in Open Biology4.931
· DOI :10.1098/RSOB.200333
Salvatore Santamaria14
Estimated H-index: 14
(Imperial College London),
Rens de Groot1
Estimated H-index: 1
(UCL: University College London),
Rens de Groot9
Estimated H-index: 9
(UCL: University College London)
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...
#1Sarah A. Stanley (ISMMS: Icahn School of Medicine at Mount Sinai)H-Index: 7
#2Zerina Balic (ISMMS: Icahn School of Medicine at Mount Sinai)H-Index: 3
Last. Dirk Hubmacher (ISMMS: Icahn School of Medicine at Mount Sinai)H-Index: 19
view all 3 authors...
Acromelic dysplasias are a group of rare musculoskeletal disorders that collectively present with short stature, pseudomuscular build, stiff joints, and tight skin. Acromelic dysplasias are caused by mutations in genes (FBN1, ADAMTSL2, ADAMTS10, ADAMTS17, LTBP2, and LTBP3) that encode secreted extracellular matrix proteins, and in SMAD4, an intracellular coregulator of transforming growth factor-β (TGF-β) signaling. The shared musculoskeletal presentations in acromelic dysplasias suggest that th...
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Aggrecan is a large proteoglycan that forms giant hydrated aggregates with hyaluronan in the extracellular matrix (ECM). The extraordinary resistance of these aggregates to compression explains their abundance in articular cartilage of joints where they ensure adequate load-bearing. In the brain, they provide mechanical buffering and contribute to formation of perineuronal nets, which regulate synaptic plasticity. Aggrecan is also present in cardiac jelly, developing heart valves, and blood vess...
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#1Tao ZengH-Index: 2
#2Jianting GanH-Index: 4
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Background: Acute aortic dissection (AAD) is associated with degeneration of the aortic media and accompanied by vascular extracellular matrix (ECM) remodeling. Recently, a disintegrin and metalloproteinase with thrombospondin type 1 motifs-5 (ADAMTS-5) has been reported to be involved in ECM remodeling and vascular diseases. The aim of this study was to examine ADAMTS-5 levels in AAD patients and investigate the underlying mechanisms. Methods: Aortic tissue samples were collected from normal do...
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#1Lei Li (Dalian Medical University)H-Index: 2
#2Wei Ma (Dalian Medical University)H-Index: 3
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Abdominal aortic aneurysm (AAA) is a serious vascular disease featured by inflammatory infiltration in aortic wall, aortic dilatation and extracellular matrix (ECM) degradation. Dysregulation of microRNAs (miRNAs) is implicated in AAA progress. By profiling miRNA expression in mouse AAA tissues and control aortas, we noted that miR-126a-5p was down-regulated by 18-fold in AAA samples, which was further validated with real-time qPCR. This study was performed to investigate miR-126a-5p's role in A...
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#1Shamima Islam (Aichi Medical University)H-Index: 15
#2Kantinan Chuensirikulchai (Aichi Medical University)H-Index: 2
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Abstract Versican is a large chondroitin sulfate/dermatan sulfate proteoglycan in the extracellular matrix, and is expressed at high levels in tissues during development and remodeling in pathological conditions. Its core protein is cleaved at a region close to the N-terminal end of CSβ domain by several members of a disintegrin and metalloproteinase with thrombospondin motifs (ADAMTS) family, i.e., ADAMTS-1, 4, 5, 9, 15, and 20. Here, using a CRISPR/Cas9 system, we generated knock-in mice (V1R)...
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#1Stylianos Z. Karoulias (ISMMS: Icahn School of Medicine at Mount Sinai)H-Index: 3
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Secreted a disintegrin-like and metalloprotease with thrombospondin type 1 motif (ADAMTS) proteases play crucial roles in tissue development and homeostasis. The biological and pathological functions of ADAMTS proteases are determined broadly by their respective substrates and their interactions with proteins in the pericellular and extracellular matrix. For some ADAMTS proteases, substrates have been identified and substrate cleavage has been implicated in tissue development and in disease. For...
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#1An-Sofie Schelpe (Katholieke Universiteit Leuven)H-Index: 6
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Plasma ADAMTS13 circulates in a folded conformation that is stabilized by an interaction between the central Spacer domain and the C-terminal CUB (complement components C1r and C1s, sea urchin protein Uegf, and bone morphogenetic protein-1) domains. Binding of ADAMTS13 to the VWF D4(-CK) domains or to certain activating murine monoclonal antibodies (mAbs) induces a structural change that extends ADAMTS13 into an open conformation that enhances its function. The objective was to characterize the ...
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#1Maria Tinajero (U of T: University of Toronto)H-Index: 1
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Abstract The adventitia, the outer layer of the blood vessel wall, may be the most complex layer of the wall and may be the master regulator of wall physiology and pathobiology. This review proposes a major shift in thinking to apply a functional lens to the adventitia rather than only a structural lens. Human and experimental in vivo and in vitro studies show that the adventitia is a dynamic micro-environment in which adventitial and perivascular adipose tissue cells initiate and regulate impor...
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Aortic aneurysm is a permanent focal dilation of the aorta. It is usually an asymptomatic disease, but can lead to sudden death due to aortic rupture. Aortic aneurysm-related mortalities are estima...
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Cited By1
#8Xiaobing Yuan (ECNU: East China Normal University)
Summary null Members of a disintegrin and metalloproteinases with thrombospondin motif (ADAMTS) family have been implicated in various vascular diseases. However, their functional roles in early embryonic vascular development are unknown. In this study, we showed that Adamts18 is highly expressed at E11.5-E14.5 in cells surrounding the embryonic aortic arch (AOAR) and the common carotid artery (CCA) during branchial arch artery development in mice. Adamts18 deficiency was found to cause abnormal...
The hyalectan family is composed of the proteoglycans aggrecan, versican, brevican and neurocan. Hyalectans, also known as lecticans, are components of the extracellular matrix of different tissues and play essential roles in key biological processes including skeletal development, and they are related to the correct maintenance of the vascular and central nervous system. For instance, hyalectans participate in the organization of structures such as perineural nets and in the regulation of neuri...
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#1George N. ChaldakovH-Index: 26
Last. Stanislav YanevH-Index: 12
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Atherosclerosis and its complications, erosion and rupture of the plaque fibrous cap, lead to myocardial infarction and stroke, the main causes of mortality worldwide. In this setting, arterial smooth muscle cells (SMC) of the innermost media undergo phenotypic changes, a switch towards a secretory phenotype engaged in matrix proteins production. In its nature, this is a protective action that forms of a new arterial layer, the fibrous cap covering the plaque thrombogenic lipid core. The risk of...