Nitric oxide mediates aortic disease in mice deficient in the metalloprotease Adamts1 and in a mouse model of Marfan syndrome

Oller, Jorge; Méndez-Barbero, Nerea; Ruiz, E Josue; Villahoz, Silvia; Renard, Marjolijn; Canelas, Lizet I; Briones, Ana M; Alberca, Rut; Lozano-Vidal, Noelia; Hurlé, María A; Milewicz, Dianna; Evangelista, Arturo; Salaices, Mercedes; Nistal, J Francisco; Jiménez-Borreguero, Luis Jesús; De Backer, Julie; Campanero, Miguel R; Redondo, Juan Miguel

doi:10.1038/nm.4266

Cited by 177Connective tissue disorders researchAortic Disease and Treatment ApproachesAortic aneurysm repair treatments

Nitric oxide mediates aortic disease in mice deficient in the metalloprotease Adamts1 and in a mouse model of Marfan syndrome

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Oller, Jorge, Méndez-Barbero, Nerea, Ruiz, E Josue, Villahoz, Silvia, Renard, Marjolijn, Canelas, Lizet I, Briones, Ana M, Alberca, Rut +10 more·2017·Nature Medicine

DOI 10.1038/nm.4266

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Abstract

Heritable thoracic aortic aneurysms and dissections (TAAD), including Marfan syndrome (MFS), currently lack a cure, and causative mutations have been identified for only a fraction of affected families. Here we identify the metalloproteinase ADAMTS1 and inducible nitric oxide synthase (NOS2) as therapeutic targets in individuals with TAAD. We show that Adamts1 is a major mediator of vascular homeostasis, given that genetic haploinsufficiency of Adamts1 in mice causes TAAD similar to MFS. Aortic nitric oxide and Nos2 levels were higher in Adamts1-deficient mice and in a mouse model of MFS (hereafter referred to as MFS mice), and Nos2 inactivation protected both types of mice from aortic pathology. Pharmacological inhibition of Nos2 rapidly reversed aortic dilation and medial degeneration in young Adamts1-deficient mice and in young or old MFS mice. Patients with MFS showed elevated NOS2 and decreased ADAMTS1 protein levels in the aorta. These findings uncover a possible causative role for the ADAMTS1-NOS2 axis in human TAAD and warrant evaluation of NOS2 inhibitors for therapy.

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Topiroxostat is a potent inhibitor of XO and induces a covalent modification in the enzyme that inactivates XO after complex dissociation.
“Topiroxostat is a potent inhibitor of XO and induces a covalent modification in the enzyme that inactivates XO after complex dissociation (629, 703, 821).”
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Marfan syndrome; A connective tissue disease at the crossroads of mechanotransduction, TGFβ signaling and cell stemness
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Critical Role of ADAMTS-4 in the Development of Sporadic Aortic Aneurysm and Dissection in Mice
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ADAMTS8 Promotes the Development of Pulmonary Arterial Hypertension and Right Ventricular Failure
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Histone methylation and vascular biology
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Extracellular Matrix Interactome in Modulating Vascular Homeostasis and Remodeling
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S-Nitrosylation of Plastin-3 Exacerbates Thoracic Aortic Dissection Formation via Endothelial Barrier Dysfunction
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Aggrecan in Cardiovascular Development and Disease
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ADAMTS‐5: A difficult teenager turning 20
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ADAR1 Non-Editing Function in Macrophage Activation and Abdominal Aortic Aneurysm
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Loss of Smooth Muscle α-Actin Leads to NF-κB–Dependent Increased Sensitivity to Angiotensin II in Smooth Muscle Cells and Aortic Enlargement
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Glycoproteomic Analysis of the Aortic Extracellular Matrix in Marfan Patients
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Molecular differences of angiogenic versus vessel co-opting colorectal cancer liver metastases at single-cell resolution
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ADAMTS9-Regulated Pericellular Matrix Dynamics Governs Focal Adhesion-Dependent Smooth Muscle Differentiation
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Pathogenic variants in THSD4, encoding the ADAMTS-like 6 protein, predispose to inherited thoracic aortic aneurysm
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Aortic pathology from protein kinase G activation is prevented by an antioxidant vitamin B12 analog
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Adamts5 −/− Mice Exhibit Altered Aggrecan Proteolytic Profiles That Correlate With Ascending Aortic Anomalies
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Reactive Oxygen Species and Oxidative Stress in the Pathogenesis and Progression of Genetic Diseases of the Connective Tissue
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Genetic Delivery and Gene Therapy in Pulmonary Hypertension
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The Emerging Role of the ADAMTS Family in Vascular Diseases
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Animal Models, Pathogenesis, and Potential Treatment of Thoracic Aortic Aneurysm
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Nitro-oleic acid reduces thoracic aortic aneurysm progression in a mouse model of Marfan syndrome
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Fibrillin-1 regulates endothelial sprouting during angiogenesis
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ADAMTS1 protease is required for a balanced immune cell repertoire and tumour inflammatory response
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Blood Flow Deficits and Cerebrovascular Changes in a Dietary Model of Hyperhomocysteinemia
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Shock waves promote spinal cord repair via TLR3
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Aortic “Disease-in-a-Dish”: Mechanistic Insights and Drug Development Using iPSC-Based Disease Modeling
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ADAMTS8 Promotes Cardiac Fibrosis Partly Through Activating EGFR Dependent Pathway
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The Multiple Functions of Fibrillin-1 Microfibrils in Organismal Physiology
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Endothelial dysfunction in Marfan syndrome mice is restored by resveratrol
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The NO signalling pathway in aortic aneurysm and dissection
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Etiology and pathogenesis of the Marfan syndrome: current understanding
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Fibrillin-2 is a key mediator of smooth muscle extracellular matrix homeostasis during mouse tracheal tubulogenesis
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Reversal of Aortic Enlargement Induced by Increased Biomechanical Forces Requires AT1R Inhibition in Conjunction With AT2R Activation
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Stimulation of Caveolin-1 Signaling Improves Arteriovenous Fistula Patency
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Visualization and Analysis of Gene Expression in Stanford Type A Aortic Dissection Tissue Section by Spatial Transcriptomics
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Postnatal deficiency of ADAMTS1 ameliorates thoracic aortic aneurysm and dissection in mice
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Inhibition of aortic CX3CR1+ macrophages mitigates thoracic aortic aneurysm progression in Marfan syndrome in mice
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Vascular dimorphism ensured by regulated proteoglycan dynamics favors rapid umbilical artery closure at birth
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Unfolding the Story of Proteoglycan Accumulation in Thoracic Aortic Aneurysm and Dissection
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Extracellular Matrix Disorganization Caused by ADAMTS16 Deficiency Leads to Bicuspid Aortic Valve With Raphe Formation
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Inflammation in Abdominal Aortic Aneurysm: Cause or Comorbidity?
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The C-terminal domains of ADAMTS1 contain exosites involved in its proteoglycanase activity
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Identification of the metalloproteinase Adamts1 and Nitric Oxide as new therapeutic targets in aortic diseases
Pedrosa, J. O. (2017) · LA Referencia (Red Federada de Repositorios Institucionales de Publicaciones Científicas)
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Postnatal deficiency of ADAMTS1 ameliorates thoracic aortic aneurysm and dissection in mice
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Authors: Oller, J., Méndez-Barbero, N., Ruiz, E. J., Villahoz, S., Renard, M., Canelas, L. I., Briones, A. M., Alberca, R., Lozano-Vidal, N., Hurlé, M. A., Milewicz, D., Evangelista, A., Salaices, M., Nistal, J. F., Jiménez-Borreguero, L. J., De Backer, J., Campanero, M. R., & Redondo, J. M.
Affiliations: Spanish National Centre for Cardiovascular Research (ES)
Ghent University Hospital (BE)
Universidad Autónoma de Madrid (ES)
Universidad de Cantabria (ES)
Vall d'Hebron Hospital Universitari (ES)
Consejo Superior de Investigaciones Científicas (ES)
Corresponding author: Campanero, Miguel R
Redondo, Juan Miguel
ORCID: Oller, Jorge
Renard, Marjolijn
Briones, Ana M
Hurlé, María A
Milewicz, Dianna
Evangelista, Arturo
Nistal, J Francisco
Jiménez-Borreguero, Luis Jesús
De Backer, Julie
Campanero, Miguel R
Redondo, Juan Miguel
Venue: Nature Medicine
Publisher: Springer Science and Business Media LLC
Published: 2017-01-09
Volume: 23
Issue: 2
Pages: 200-212
ISSN: 1078-8956 (ISSN-L)
1546-170X
DOI: 10.1038/nm.4266
OpenAlex: W2569369624
Cited by: 177
References: 59
Citations by year: 2026: 6 · 2025: 23 · 2024: 17 · 2023: 21 · 2022: 14 · 2021: 22 · 2020: 20 · 2019: 20 · 2018: 18 · 2017: 14 · 2015: 1
FWCI: 13.07
Type: journal article
Language: en
Open access: green
hdl.handle.net
Licence: cc-by-nc-sa
www.springer.com/tdm (tdm)
Locations: Nature Medicine (publishedVersion)
PubMed (publishedVersion)
Ghent University Academic Bibliography (Ghent University) (submittedVersion)
DIGITAL.CSIC (Spanish National Research Council (CSIC)) (submittedVersion)
LA Referencia (Red Federada de Repositorios Institucionales de Publicaciones Científicas) (submittedVersion, cc-by-nc-sa)
Topics: Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Connective tissue disorders research
Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Aortic Disease and Treatment Approaches
Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Aortic aneurysm repair treatments
Keywords: ADAMTS1, inducible nitric oxide synthase, nitric oxide, Marfan syndrome, thoracic aortic aneurysm, aortic dissection, aortic disease, metalloproteinases, vascular homeostasis, aortic dilatation, medial degeneration, therapeutic target
MeSH terms: ADAMTS1 Protein, Fibrillin-1, Adult, Aged, Aortic Dissection, Animals, Aorta, Aortic Aneurysm, Disease Models, Animal, Enzyme Inhibitors, Female, Humans, Male, Marfan Syndrome, Middle Aged, Nitric Oxide, Immunoblotting, Aortic Aneurysm, Thoracic, NG-Nitroarginine Methyl Ester, Mice, Nitric Oxide Synthase Type II, Adipokines, Gene Knockdown Techniques, Haploinsufficiency, Real-Time Polymerase Chain Reaction

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Nitric oxide mediates aortic disease in mice deficient in the metalloprotease Adamts1 and in a mouse model of Marfan syndrome

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