ArticleMatrix biology : journal of the International Society for Matrix Biology2016
Characterization of metabolic health in mouse models of fibrillin-1 perturbation.
Article in Matrix biology : journal of the International Society for Matrix Biology, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 10 papers, 1 of them a synthesis that pooled it.
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Who cites it
10 citing papers in PubMed, 1 synthesis or guideline pooled it, 18 citations in OpenAlex.
- Skeletal muscle alterations in Marfan syndrome: a systematic review.Journal of muscle research and cell motility · 2025Pooled it
- Tissue material properties, whole-bone morphology and mechanical behavior in theMatrix biology plus · 2024Article
- Unique Features of Cardiovascular Involvement and Progression in Children with Marfan Syndrome Justify Dedicated Multidisciplinary Care.Journal of cardiovascular development and disease · 2024Review
- MFAP2 promotes HSCs activation through FBN1/TGF-β/Smad3 pathway.Journal of cellular and molecular medicine · 2023Article
- The extracellular matrix glycoprotein fibrillin-1 in health and disease.Frontiers in cell and developmental biology · 2023Review
- Fibrillin-1 and fibrillin-1-derived asprosin in adipose tissue function and metabolic disorders.Journal of cell communication and signaling · 2020Review
- Overexpressed MAGP1 Is Associated With a Poor Prognosis and Promotes Cell Migration and Invasion in Gastric Cancer.Frontiers in oncology · 2019Article
- Microfibril-associated glycoproteins MAGP-1 and MAGP-2 in disease.Matrix biology : journal of the International Society for Matrix Biology · 2018Review
- Structural and compositional diversity of fibrillin microfibrils in human tissues.The Journal of biological chemistry · 2018Article
- Contribution of metabolic disease to bone fragility in MAGP1-deficient mice.Matrix biology : journal of the International Society for Matrix Biology · 2018Article
Corrections and comments
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Authors and funding
6 authors at 2 institutions in 1 country.
Funding
Abstract
Mutations in the microfibrillar protein fibrillin-1 or the absence of its binding partner microfibril-associated glycoprotein (MAGP1) lead to increased TGFβ signaling due to an inability to sequester latent or active forms of TGFβ, respectively. Mouse models of excess TGFβ signaling display increased adiposity and predisposition to type-2 diabetes. It is therefore interesting that individuals with Marfan syndrome, a disease in which fibrillin-1 mutation leads to aberrant TGFβ signaling, typically present with extreme fat hypoplasia. The goal of this project was to characterize multiple fibrillin-1 mutant mouse strains to understand how fibrillin-1 contributes to metabolic health. The results of this study demonstrate that fibrillin-1 contributes little to lipid storage and metabolic homeostasis, which is in contrast to the obesity and metabolic changes associated with MAGP1 deficiency. MAGP1 but not fibrillin-1 mutant mice had elevated TGFβ signaling in their adipose tissue, which is consistent with the difference in obesity phenotypes. However, fibrillin-1 mutant strains and MAGP1-deficient mice all exhibit increased bone length and reduced bone mineralization which are characteristic of Marfan syndrome. Our findings suggest that Marfan-associated adipocyte hypoplasia is likely not due to microfibril-associated changes in adipose tissue, and provide evidence that MAGP1 may function independently of fibrillin in some tissues.
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Registered trials
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