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animals (13) 13
humans (13) 13
mice (12) 12
disease models, animal (10) 10
mutation (10) 10
phenotype (10) 10
fibrillins (9) 9
growth-factor-beta (9) 9
microfilament proteins - genetics (9) 9
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marfan syndrome - pathology (8) 8
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medicine, research & experimental (5) 5
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genetics & heredity (4) 4
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aneurysms (3) 3
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cardiac & cardiovascular systems (3) 3
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congenital, hereditary, and neonatal diseases and abnormalities (3) 3
elastic tissue - pathology (3) 3
genes (3) 3
health aspects (3) 3
immunohistochemistry (3) 3
in-vivo (3) 3
marfan syndrome - immunology (3) 3
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receptors, transforming growth factor beta - genetics (3) 3
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abnormalities, multiple (2) 2
abridged index medicus (2) 2
actins - analysis (2) 2
alleles (2) 2
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angiotensin ii type 1 receptor blockers - therapeutic use (2) 2
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aorta - cytology (2) 2
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loeys-dietz syndrome - genetics (2) 2
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Journal of the American College of Cardiology, ISSN 0735-1097, 2015, Volume 65, Issue 13, pp. 1324 - 1336
Journal Article
Nature Genetics, ISSN 1061-4036, 2012, Volume 44, Issue 8, pp. 922 - 927
Journal Article
Science, ISSN 0036-8075, 4/2006, Volume 312, Issue 5770, pp. 117 - 121
Aortic aneurysm and dissection are manifestations of Marfan syndrome (MFS), a disorder caused by mutations in the gene that encodes fibrillin-1. Selected... 
Airspace | Receptors | Architecture | Dilatation | Root growth | Placebos | Reports | Mice | Aortic aneurysm | Elastic tissue | Marfan syndrome | PATHOGENESIS | GROWTH-FACTOR-BETA | ACTIVATION | FIBRILLIN | MULTIDISCIPLINARY SCIENCES | RATS | RECEPTOR | SMOOTH-MUSCLE-CELLS | BLOCKADE | CONTRIBUTES | DILATATION | Angiotensin II Type 1 Receptor Blockers - therapeutic use | Propranolol - therapeutic use | Neutralization Tests | Aortic Aneurysm - etiology | Lung Diseases - drug therapy | Marfan Syndrome - drug therapy | Propranolol - administration & dosage | Antibodies - immunology | Transforming Growth Factor beta - antagonists & inhibitors | Female | Marfan Syndrome - complications | Microfilament Proteins - genetics | Adrenergic beta-Antagonists - administration & dosage | Adrenergic beta-Antagonists - therapeutic use | Angiotensin II Type 1 Receptor Blockers - administration & dosage | Aortic Aneurysm - prevention & control | Disease Models, Animal | Transforming Growth Factor beta - immunology | Fibrillin-1 | Lung - pathology | Pulmonary Alveoli - pathology | Signal Transduction | Fibrillins | Pregnancy Complications - drug therapy | Aorta - pathology | Pregnancy | Elastic Tissue - pathology | Animals | Receptor, Angiotensin, Type 1 - metabolism | Losartan - therapeutic use | Marfan Syndrome - metabolism | Marfan Syndrome - pathology | Losartan - administration & dosage | Lung Diseases - pathology | Mutation | Transforming Growth Factor beta - metabolism | Complications and side effects | Aortic aneurysms | Genetic aspects | Research | Risk factors
Journal Article
Journal Article
Journal of Clinical Investigation, ISSN 0021-9738, 01/2014, Volume 124, Issue 1, pp. 448 - 460
Loeys-Dietz syndrome (LDS) is a connective tissue disorder that is characterized by a high risk for aneurysm and dissection throughout the arterial tree and... 
Connective tissue diseases | Angiotensin | Physiological aspects | Development and progression | Genetic aspects | Cellular signal transduction | Research | Transforming growth factors | Studies | Substance abuse treatment | Rodents | Genes | Cloning | Mutation | Kinases | Dissection | Age
Journal Article
JOURNAL OF CLINICAL INVESTIGATION, ISSN 0021-9738, 01/2014, Volume 124, Issue 1, pp. 448 - 460
Journal Article
Journal Article
European Journal of Human Genetics, ISSN 1018-4813, 02/2015, Volume 23, Issue 2, pp. 224 - 228
Journal Article
Science Translational Medicine, ISSN 1946-6234, 07/2013, Volume 5, Issue 195
Journal Article
Journal Article
Journal of the American College of Cardiology, ISSN 0735-1097, 04/2015, Volume 65, Issue 13, pp. 1324 - 1336
markdownabstractBACKGROUND Aneurysms affecting the aorta are a common condition associated with high mortality as a result of aortic dissection or rupture.... 
Immunohistochemistry | Aneurysm, Dissecting - genetics | Genetic Predisposition to Disease | Genome-Wide Association Study | Transforming Growth Factor beta3 - genetics | Humans | Middle Aged | Genotype | Male | Sequence Analysis, DNA | Pedigree | Electrocardiography | Adult | Female | Aged | Mutation | Aortic Aneurysm - genetics
Journal Article
Circulation Research: Journal of the American Heart Association, ISSN 0009-7330, 01/2001, Volume 88, Issue 1, pp. 37 - 43
ABSTRACT—Marfan syndrome is associated with early death due to aortic aneurysm. The condition is caused by mutations in the gene (FBN1) encoding fibrillin-1, a... 
Fibrillin | Elastin | Marfan syndrome | Aneurysm | Microfibril | aneurysm | MOLECULAR-BIOLOGY | CARDIAC & CARDIOVASCULAR SYSTEMS | ABDOMINAL AORTIC-ANEURYSMS | ELASTIC LAMINA | elastin | microfibril | SPONTANEOUSLY HYPERTENSIVE RATS | MATRIX METALLOPROTEINASES | GENE | FIBRILLIN-1 | PERIPHERAL VASCULAR DISEASE | HEMATOLOGY | CATHEPSIN-D | EXPRESSION | BINDING | fibrillin
Journal Article