ArticleHuman molecular genetics2010
Differential effects of alendronate and losartan therapy on osteopenia and aortic aneurysm in mice with severe Marfan syndrome.
Article in Human molecular genetics, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 28 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
28 citing papers in PubMed, 77 citations in OpenAlex.
- The aorta in Marfan syndrome: from molecular mechanisms to mechanobiological dysfunction.Cardiovascular research · 2026Review
- Tissue material properties, whole-bone morphology and mechanical behavior in theMatrix biology plus · 2024Article
- Exploration of key factors in Gingival Crevicular fluids from patients undergoing Periodontally Accelerated Osteogenic Orthodontics (PAOO) using proteome analysis.BMC oral health · 2023Article
- Fibrillin-1 deficiency in the outer perichondrium causes longitudinal bone overgrowth in mice with Marfan syndrome.Human molecular genetics · 2022Article
- The CMS19 disease model specifies a pivotal role for collagen XIII in bone homeostasis.Scientific reports · 2022Article
- Assessment of Bones Deficient in Fibrillin-1 Microfibrils Reveals Pronounced Sex Differences.International journal of molecular sciences · 2019Article
- Marfan syndrome; A connective tissue disease at the crossroads of mechanotransduction, TGFβ signaling and cell stemness.Matrix biology : journal of the International Society for Matrix Biology · 2018Review
- LTBP3 Pathogenic Variants Predispose Individuals to Thoracic Aortic Aneurysms and Dissections.American journal of human genetics · 2018Article
- The Effect of a Nonpeptide Angiotensin II Type 2 Receptor Agonist, Compound 21, on Aortic Aneurysm Growth in a Mouse Model of Marfan Syndrome.Journal of cardiovascular pharmacology · 2018Article
- Angiotensin, transforming growth factor β and aortic dilatation in Marfan syndrome: Of mice and humans.International journal of cardiology. Heart & vasculature · 2018Article
- Examining tissue composition, whole-bone morphology and mechanical behavior of GorabJournal of biomechanics · 2017Article
- Losartan Attenuates Degradation of Aorta and Lung Tissue Micromechanics in a Mouse Model of Severe Marfan Syndrome.Annals of biomedical engineering · 2016Article
- Insights into ascending aortic aneurysm pathogenesis using in vivo and ex vivo imaging systems in angiotensin II-infused mice.Journal of thoracic disease · 2016Article
- Generation of heterozygous fibrillin-1 mutant cloned pigs from genome-edited foetal fibroblasts.Scientific reports · 2016Article
- Fibrillin-1 Regulates Skeletal Stem Cell Differentiation by Modulating TGFβ Activity Within the Marrow Niche.Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research · 2016Article
- The microfibril-associated glycoproteins (MAGPs) and the microfibrillar niche.Matrix biology : journal of the International Society for Matrix Biology · 2015Review
- Dimorphic effects of transforming growth factor-β signaling during aortic aneurysm progression in mice suggest a combinatorial therapy for Marfan syndrome.Arteriosclerosis, thrombosis, and vascular biology · 2015Article
- The ever-expanding conundrum of primary osteoporosis: aetiopathogenesis, diagnosis, and treatment.Italian journal of pediatrics · 2014Review
- Microfibril-associated glycoprotein 2 (MAGP2) loss of function has pleiotropic effects in vivo.The Journal of biological chemistry · 2013Article
- Fibrillin-1 directly regulates osteoclast formation and function by a dual mechanism.Journal of cell science · 2013Article
Corrections and comments
- Erratum issued
Authors and funding
10 authors at 3 institutions in 1 country.
Funding
Abstract
Reduced bone mineral density (osteopenia) is a poorly characterized manifestation of pediatric and adult patients afflicted with Marfan syndrome (MFS), a multisystem disorder caused by structural or quantitative defects in fibrillin-1 that perturb tissue integrity and TGFβ bioavailability. Here we report that mice with progressively severe MFS (Fbn1(mgR/mgR) mice) develop osteopenia associated with normal osteoblast differentiation and bone formation. In vivo and ex vivo experiments, respectively, revealed that adult Fbn1(mgR/mgR) mice respond more strongly to locally induced osteolysis and that Fbn1(mgR/mgR) osteoblasts stimulate pre-osteoclast differentiation more than wild-type cells. Greater osteoclastogenic potential of mutant osteoblasts was largely attributed to Rankl up-regulation secondary to improper TGFβ activation and signaling. Losartan treatment, which lowers TGFβ signaling and restores aortic wall integrity in mice with mild MFS, did not mitigate bone loss in Fbn1(mgR/mgR) mice even though it ameliorated vascular disease. Conversely, alendronate treatment, which restricts osteoclast activity, improved bone quality but not aneurysm progression in Fbn1(mgR/mgR) mice. Taken together, our findings shed new light on the pathogenesis of osteopenia in MFS, in addition to arguing for a multifaceted treatment strategy in this congenital disorder of the connective tissue.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.