Aortic microcalcification is associated with elastin fragmentation in Marfan syndrome

Marfan syndrome (MFS) is a connective tissue disorder in which aortic rupture is the major cause of death. MFS patients with an aortic diameter below the advised limit for prophylactic surgery (

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Bibliographic Details
Published in:The Journal of pathology 2017-11, Vol.243 (3), p.294-306
Main Authors: Wanga, Shaynah, Hibender, Stijntje, Ridwan, Yanto, van Roomen, Cindy, Vos, Mariska, van der Made, Ingeborg, van Vliet, Nicole, Franken, Romy, van Riel, Luigi AMJG, Groenink, Maarten, Zwinderman, Aeilko H, Mulder, Barbara JM, de Vries, Carlie JM, Essers, Jeroen, de Waard, Vivian
Format: Article
Language:English
Subjects:
Alkaline phosphatase
aneurysm
Aneurysms
Animals
Aorta
Aorta - metabolism
Aortic Aneurysm - metabolism
Aortic Aneurysm - pathology
Calcification
Calcification (ectopic)
Calcinosis - metabolism
Dissection
Elastin
Elastin - metabolism
Enzyme inhibitors
Exo-a-sialidase
Extracellular signal-regulated kinase
Extracellular Signal-Regulated MAP Kinases - metabolism
Fragmentation
Fragments
Humans
I.R. radiation
Kinases
Lactose
MAP kinase
Marfan syndrome
Marfan Syndrome - metabolism
Marfan Syndrome - pathology
Mice
Mitogen-Activated Protein Kinase Kinases - metabolism
mRNA
Muscle, Smooth, Vascular - metabolism
Muscle, Smooth, Vascular - pathology
Myocytes, Smooth Muscle - metabolism
Myocytes, Smooth Muscle - pathology
Peptides
Phosphorylation
Protein kinase
Rupture
Smooth muscle
Surgery
transgenic mice
vascular calcification
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Description
Summary:Marfan syndrome (MFS) is a connective tissue disorder in which aortic rupture is the major cause of death. MFS patients with an aortic diameter below the advised limit for prophylactic surgery (
ISSN:0022-3417
1096-9896
DOI:10.1002/path.4949