Fibrillin-1 and -2 differentially modulate endogenous TGF-β and BMP bioavailability during bone formation

Extracellular microfibrils composed of fibrillin-1 and -2 regulate bone formation through modulation of TGF-β and BMP signaling.

• Publication year

  2010

• Venue

  Journal of Cell Biology

• Publication date

  2010-09-20

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1083/jcb.201003089PMID 20855508PMCID 3101602
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• Microfibril Structure Masks Fibrillin-2 in Postnatal Tissues*

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• Identification of a frameshift mutation in Osterix in a patient with recessive osteogenesis imperfecta.

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• Extracellular Microfibrils Control Osteoblast-supported Osteoclastogenesis by Restricting TGFβ Stimulation of RANKL Production*

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• New regulatory mechanisms of TGF-beta receptor function.

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• Latent Transforming Growth Factor β-binding Proteins and Fibulins Compete for Fibrillin-1 and Exhibit Exquisite Specificities in Binding Sites*

  2009influential reference
• Genetic control of bone formation.

  2009cited by this paper
• p38 MAPK Is an Early Determinant of Promiscuous Smad2/3 Signaling in the Aortas of Fibrillin-1 (Fbn1)-null Mice*

  2009influential reference
• Biogenesis and function of fibrillin assemblies

  2009cited by this paper
• Extracellular microfibrils: contextual platforms for TGFbeta and BMP signaling.

  2009cited by this paper
• Angiotensin II blockade and aortic-root dilation in Marfan's syndrome.

  2008cited by this paper
• Marfan syndrome and related disorders.

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• A new model for growth factor activation: type II receptors compete with the prodomain for BMP-7.

  2008influential reference
• Biogenesis of extracellular microfibrils: Multimerization of the fibrillin-1 C terminus into bead-like structures enables self-assembly

  2008cited by this paper
• 5 The Bone Morphogenetic Proteins

  2008cited by this paper
• Fibrillin assembly requires fibronectin.

  2008cited by this paper
• Targeting of Bone Morphogenetic Protein Growth Factor Complexes to Fibrillin*

  2008influential reference
• Extracellular control of TGFβ signalling in vascular development and disease

  2007cited by this paper
• Comparative transcriptome analysis of embryonic and adult stem cells with extended and limited differentiation capacity

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• Potential role for heparan sulfate proteoglycans in regulation of transforming growth factor-beta (TGF-beta) by modulating assembly of latent TGF-beta-binding protein-1.

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• Extracellular control of TGFbeta signalling in vascular development and disease.

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• Marfan syndrome: from molecular pathogenesis to clinical treatment.

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• 22 TGF-β Family Signaling in Skeletal Development, Maintenance, and Disease

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• Potential Role for Heparan Sulfate Proteoglycans in Regulation of Transforming Growth Factor-β (TGF-β) by Modulating Assembly of Latent TGF-β-binding Protein-1*

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• A rapid and sensitive bioassay to measure bone morphogenetic protein activity

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• An assay for the determination of biologically active bone morphogenetic proteins using cells transfected with an inhibitor of differentiation promoter-luciferase construct.

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• p53 functions as a negative regulator of osteoblastogenesis, osteoblast-dependent osteoclastogenesis, and bone remodeling

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• Losartan, an AT1 Antagonist, Prevents Aortic Aneurysm in a Mouse Model of Marfan Syndrome

  2006influential reference
• Fibrillins 1 and 2 Perform Partially Overlapping Functions during Aortic Development*

  2006influential reference
• Distinct roles for Hedgehog and canonical Wnt signaling in specification, differentiation and maintenance of osteoblast progenitors

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• Expression Profile of Osteoblast Lineage at Defined Stages of Differentiation*

  2005influential reference
• The Prodomain of BMP-7 Targets the BMP-7 Complex to the Extracellular Matrix*

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• Fibronectin regulates latent transforming growth factor-beta (TGF beta) by controlling matrix assembly of latent TGF beta-binding protein-1.

  2005cited by this paper
• Fibronectin Regulates Latent Transforming Growth Factor-β (TGFβ) by Controlling Matrix Assembly of Latent TGFβ-binding Protein-1*

  2005influential reference
• Unique coexpression in osteoblasts of broadly expressed genes accounts for the spatial restriction of ECM mineralization to bone.

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• Short protocols in cell biology : a compendium of methods from Current protocols in cell biology

  2004cited by this paper
• Evidence for a critical contribution of haploinsufficiency in the complex pathogenesis of Marfan syndrome.

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• Bone Morphogenetic Proteins

  2004influential reference
• Endogenous TGF‐β signaling suppresses maturation of osteoblastic mesenchymal cells

  2004cited by this paper
• Making sense of latent TGFβ activation

  2003cited by this paper
• Mechanisms of TGF-beta signaling from cell membrane to the nucleus.

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• Dysregulation of TGF-beta activation contributes to pathogenesis in Marfan syndrome.

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• Mechanisms of TGF-β Signaling from Cell Membrane to the Nucleus

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• Latent Transforming Growth Factor β-binding Protein 1 Interacts with Fibrillin and Is a Microfibril-associated Protein*

  2003influential reference
• Fibrillins Can Co-assemble in Fibrils, but Fibrillin Fibril Composition Displays Cell-specific Differences*

  2003cited by this paper
• Making sense of latent TGFbeta activation.

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• TGFp Signals through a Heteromeric Protein Kinase Receptor Complex

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• Identification of genes regulated during osteoblastic differentiation by genome-wide expression analysis of mouse calvaria primary osteoblasts in vitro.

  2003cited by this paper
• Fibrillin-1 and fibrillin-2 in human embryonic and early fetal development.

  2002cited by this paper
• The novel zinc finger-containing transcription factor osterix is required for osteoblast differentiation and bone formation.

  2002influential reference
• Regulation of limb patterning by extracellular microfibrils

  2001influential reference
• TGF‐β‐induced repression of CBFA1 by Smad3 decreases cbfa1 and osteocalcin expression and inhibits osteoblast differentiation

  2001cited by this paper
• Expression of fibrillins and other microfibril-associated proteins in human bone and osteoblast-like cells.

  2000cited by this paper
• Pathogenetic sequence for aneurysm revealed in mice underexpressing fibrillin-1.

  1999cited by this paper
• Osf2/Cbfa1: a transcriptional activator of osteoblast differentiation.

  1997cited by this paper
• Developmental expression of fibrillin genes suggests heterogeneity of extracellular microfibrils

  1995cited by this paper
• The effects of cytokines and growth factors on osteoblastic cells.

  1995influential reference
• Structure and expression of fibrillin-2, a novel microfibrillar component preferentially located in elastic matrices

  1994cited by this paper
• An assay for transforming growth factor-beta using cells transfected with a plasminogen activator inhibitor-1 promoter-luciferase construct

  1994influential reference
• An assay for transforming growth factor-beta using cells transfected with a plasminogen activator inhibitor-1 promoter-luciferase construct.

  1994cited by this paper
• TGF beta signals through a heteromeric protein kinase receptor complex.

  1992cited by this paper
• The BMP proteins in bone formation and repair.

  1992cited by this paper
• Bone cell differentiation: a functionally coupled relationship between expression of cell-growth- and tissue-specific genes.

  1990cited by this paper
• Transforming growth factor beta is a bifunctional regulator of replication and collagen synthesis in osteoblast-enriched cell cultures from fetal rat bone.

  1987cited by this paper
• Mineralized bone nodules formedin vitro from enzymatically released rat calvaria cell populations

  1986cited by this paper

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