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Extracellular matrixRegulating microfibril assemblyElastinFBN1GlycoproteinProteinConnective tissuesMutationsADAMTSSpecificityInteractsEpidermalBundlesRegulatesStructuralFragmentMeSHSkeletalMechanismInteractionBovineOrganizationFormationCellsMolecularAssemblyBindOrientationProvideCellAffectForceInclude

Extracellular matrix3

• Human fibrillin-1, the major structural protein of extracellular matrix (ECM) 10-12 nm microfibrils, is dominated by 43 calcium binding epidermal growth factor-like (cbEGF) and 7 transforming growth factor beta binding protein-like (TB) domains. (nih.gov)
• The human extracellular matrix glycoprotein fibrillin-1 is the primary component of the 10- to 12-nm-diameter microfibrils, which perform key structural and regulatory roles in connective tissues. (eurekamag.com)
• Components of the extracellular matrix consisting primarily of fibrillin. (bvsalud.org)

Regulating microfibril assembly1

• These data suggest that heparan sulfate may sequester fibrillin at the cell surface via FUN-EGF3 prior to aggregation of the C terminus, thereby regulating microfibril assembly. (eurekamag.com)

Elastin10

• In the formation of elastic fiber, fibrillin microfibrils guides the deposit of tropoelastin and remains in the outer layer of mature elastin fibers. (wikipedia.org)
• Elastic fibers are composed of the protein elastin and a network of 10-12 nm microfibrils . (bvsalud.org)
• FBN1 Fibrillins are structural components of 10-12 nm extracellular calcium-binding microfibrils, which occur either in association with elastin or in elastin-free bundles. (affbiotech.com)
• Microfibrils, elastin fibres and collagen fibres in the human intervertebral disc and bovine tail disc. (ox.ac.uk)
• Co-localization of microfibrils and elastin fibres was examined by dual immunostaining of fibrillin-1 and elastin. (ox.ac.uk)
• In addition, the microfibril bundles were orientated parallel to each other and co-localized highly with elastin fibres. (ox.ac.uk)
• Within each lamella, co-localized microfibrils and elastin fibres were aligned in the same direction as the collagen fibres. (ox.ac.uk)
• The microfibrils in this region co-localized with elastin fibres far less than in the OAF. (ox.ac.uk)
• In nucleus pulposus, filamentous microfibrils were organized mainly around the cells where elastin fibres were hardly detected. (ox.ac.uk)
• By contrast, sparse elastin fibres, with a few of microfibrils, were visible in the interterritorial matrix. (ox.ac.uk)

FBN12

• WMS can also be caused by mutations in fibrillin-1 (FBN1), which suggests that ADAMTS10 and ADAMTS17 cooperate with fibrillin-1 in a common biological pathway during tissue development and homeostasis. (encyclopedia.pub)
• Mutations in the FBN1 locus of the fibrillin gene on chromosome 15 have been linked to MFS and other distinct clinical entities with similar findings. (medscape.com)

Glycoprotein2

• Microfibril-associated glycoprotein-2 interacts with fibrillin-1 and fibrillin-2 suggesting a role for MAGP-2 in elastic fiber assembly. (bvsalud.org)
• ADAMTSL4, a secreted glycoprotein widely distributed in the eye, binds fibrillin-1 microfibrils and accelerates microfibril biogenesis. (medlineplus.gov)

Protein7

• In Marfan syndrome, a connective tissue disorder, mutations in the gene encoding for the fibrillin-1 protein impact nearly every one of its domains. (wikipedia.org)
• In this matrix, the ADAMTSL4 protein attaches (binds) to another protein called fibrillin-1. (medlineplus.gov)
• A lack of functional ADAMTSL4 protein likely diminishes the ability of fibrillin-1 to effectively form microfibrils. (medlineplus.gov)
• Sakai et al identified fibrillin, a 350-kd protein, by using monoclonal antibodies raised against myofibrils. (medscape.com)
• It has a structural and functional homology with matrix protein fibrillin-1 and fibrillin-2. (org.pk)
• The abnormality of the connective tissue protein fibrillin causes a variety of problems in the affected individual. (medscape.com)
• Fibrillin's structure and function are altered by abnormal protein folding due to the alteration of bonding between cysteine residues, which in turn causes defective microfibril production. (medscape.com)

Connective tissues1

• Fibrillin microfibrils are found in connective tissues, which mainly makes up fibrillin-1 and provides elasticity. (wikipedia.org)

Mutations3

• Similar to isolated ectopia lentis, the ADAMTSL4 gene mutations that cause ectopia lentis et pupillae lead to decreased production of microfibrils or the formation of impaired microfibrils, which prevents the proper anchoring of certain structures in the eyes. (medlineplus.gov)
• Several point mutations have now been identified in the fibrillin gene, most of which affect cysteine residues within the microfibril. (medscape.com)
• Thus, these mutations are thought to cause defective fibrillin to be produced. (medscape.com)

ADAMTS3

• Genetic and functional linkage between ADAMTS superfamily proteins and fibrillin-1: a novel mechanism influencing microfibril assembly and function. (medlineplus.gov)
• Hubmacher, D. The ADAMTS/Fibrillin Connection. (encyclopedia.pub)
• The ADAMTS/Fibrillin Connection" Encyclopedia , https://encyclopedia.pub/entry/10926 (accessed December 10, 2023). (encyclopedia.pub)

Specificity1

• The MAGP-2 bait was unable to interact productively with other epidermal growth factor repeats in fibrillin-1 , demonstrating specificity of the interaction. (bvsalud.org)

Interacts1

• These results demonstrate that MAGP-2 specifically interacts with fibrillin-1 and -2 and suggest that MAGP-2 may help regulate microfibrillar assembly. (bvsalud.org)

Epidermal2

• Here, we present the structure of a fibrillin N-terminal fragment comprising the fibrillin unique N-terminal (FUN) and the first three epidermal growth factor (EGF)-like domains (FUN-EGF3). (eurekamag.com)
• Deletion analysis of partial fibrillin-1 and -2 clones revealed a calcium -binding epidermal growth factor repeat-containing region near the C terminus responsible for binding. (bvsalud.org)

Bundles1

• In the outer annulus fibrosus (OAF), abundant microfibrils organized in bundles were mainly distributed in the interterritorial matrix. (ox.ac.uk)

Regulates3

• Fibrillin-1 regulates endothelial sprouting during angiogenesis. (mcgill.ca)
• Fibrillin-1 regulates arteriole integrity in the retina. (mcgill.ca)
• 2022). Fibrillin-1 regulates white adipose tissue development, homeostasis, and function. (mcgill.ca)

Structural1

• Fibrillin-1- containing microfibrils provide long-term force bearing structural support. (affbiotech.com)

Fragment1

• Crystal structures reveal the integrin binding cbEGF22-TB4-cbEGF23 fragment of human fibrillin-1 to be a Ca(2+)-rigidified tetragonal pyramid. (nih.gov)

MeSH1

• In the inner annulus fibrosus, the microfibrils were predominantly visible as a filamentous mesh network, both in the interterritorial matrix and also around the cells. (ox.ac.uk)

Skeletal1

• 2022). Proteolysis of fibrillin-2 microfibrils is essential for normal skeletal development. (mcgill.ca)

Mechanism1

• In light of our data, we propose a novel model for the assembly of the fibrillin microfibril and a mechanism to explain its extensibility. (nih.gov)

Interaction2

• Modeling, cell attachment and spreading assays, immunocytochemistry, and surface plasmon resonance indicate that cbEGF22 bound to TB4 is a requirement for integrin activation and provide insight into the molecular basis of the fibrillin-1 interaction with alphaVbeta3. (nih.gov)
• Studies using recombinant fibrillin fragments indicate that an interaction between the N- and C-terminal regions drives head-to-tail assembly. (eurekamag.com)

Bovine1

• The distribution of microfibrils was studied immunohistochemically in intervertebral discs taken from young normal human surgical cases and from the bovine tail. (ox.ac.uk)

Organization1

• The organization of microfibrils forming the primary cell wall is rather disorganized. (wikipedia.org)

Formation2

• Formation of fibrillin microfibrils in the pericellular region affects the activity of a growth factor called TGFβ. (wikipedia.org)
• 2022). Fibrillin-1 regulated miR-122 has a critical role in thoracic aortic aneurysm formation. (mcgill.ca)

Cells1

• Immunoprecipitation of MAGP-2 from transfected COS-7 cells resulted in the coprecipitation of fibrillin . (bvsalud.org)

Molecular1

• Relatively little is known about the molecular mechanisms of fibrillin assembly into microfibrils. (eurekamag.com)

Assembly2

• Assembly of fibrillin microfibrils governs extracellular deposition of latent TGFβ" (PDF). (wikipedia.org)
• It is likely that the binding of ADAMTSL4 to fibrillin-1 promotes microfibril assembly. (medlineplus.gov)

Bind2

• This region is distinct from the region of fibrillin-1 reported by others to bind MAGP-1. (bvsalud.org)
• Fibrillin-1 proteins bind to each other and other proteins to form threadlike filaments called microfibrils. (medlineplus.gov)

Orientation1

• We suggest that other cbEGF-TB pairs within the fibrillins may adopt a similar orientation to cbEGF22-TB4. (nih.gov)

Provide1

• Microfibrils provide support to many tissues, including the lenses of the eyes, which are held in their central position by these filaments. (medlineplus.gov)

Cell4

• Cellulose microfibrils are laid down in the inner surface of the primary cell wall. (wikipedia.org)
• As the cell absorbs water, its volume increases and the existing microfibrils separate and new ones are formed to help increase cell strength. (wikipedia.org)
• Eventually this results in the cell becoming wrapped in a microfibril layer. (wikipedia.org)
• The microfibril is also associated in cell communication. (wikipedia.org)

Affect1

• Such defects in fibrillin-1 affect the signaling of TGFβ, as microfibrils directly govern the activity of TGFβ. (wikipedia.org)

Force1

• These lanes force microfibrils to remain in a certain area while they wrap. (wikipedia.org)

Include1

• The microfibrillar proteins include, among others, the fibrillins and microfibril -associated glycoproteins -1 and -2 (MAGP-1 and MAGP-2). (bvsalud.org)