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>   home   >   Products   >   Primary Antibodies   >   Signal Transduction   >   FBN1 / Fibrillin 1 Antibody (clone 3H6)   

FBN1 / Fibrillin 1 Antibody (clone 3H6)

Mouse Monoclonal Antibody

     
  • IHC - FBN1 / Fibrillin 1 Antibody (clone 3H6) ALS14370
    Anti-FBN1 / Fibrillin 1 antibody IHC of human placenta.
  • SPECIFICATION
  • CITATIONS
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  • BACKGROUND
Product Information
Application
  • Applications Legend:
  • WB=Western Blot
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin-embedded Sections)
  • IHC-F=Immunohistochemistry (Frozen Sections)
  • IF=Immunofluorescence
  • FC=Flow Cytopmetry
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • E=ELISA
  • IP=Immunoprecipitation
  • DB=Dot Blot
  • CHIP=Chromatin Immunoprecipitation
  • FA=Fluorescence Assay
  • IEM=Immunoelectronmicroscopy
  • EIA=Enzyme Immunoassay
WB, IHC-P, E
Primary Accession P35555
Reactivity Human
Host Mouse
Clonality Monoclonal
Clone Names 3H6
Calculated MW 312kDa
Dilution IHC-P (5 µg/ml), WB (1:500-1:1000),
Additional Information
Gene ID 2200
Other Names Fibrillin-1, FBN1, FBN
Target/Specificity Human Fibrillin 1
Reconstitution & Storage Aliquot and store at -20°C or -80°C. Avoid freeze-thaw cycles.
PrecautionsFBN1 / Fibrillin 1 Antibody (clone 3H6) is for research use only and not for use in diagnostic or therapeutic procedures.
Protein Information
Name FBN1
Synonyms FBN
Function Fibrillin-1: Structural component of the 10-12 nm diameter microfibrils of the extracellular matrix, which conveys both structural and regulatory properties to load-bearing connective tissues (PubMed:1860873, PubMed:15062093). Fibrillin-1- containing microfibrils provide long-term force bearing structural support. In tissues such as the lung, blood vessels and skin, microfibrils form the periphery of the elastic fiber, acting as a scaffold for the deposition of elastin. In addition, microfibrils can occur as elastin-independent networks in tissues such as the ciliary zonule, tendon, cornea and glomerulus where they provide tensile strength and have anchoring roles. Fibrillin-1 also plays a key role in tissue homeostasis through specific interactions with growth factors, such as the bone morphogenetic proteins (BMPs), growth and differentiation factors (GDFs) and latent transforming growth factor-beta-binding proteins (LTBPs), cell- surface integrins and other extracellular matrix protein and proteoglycan components (PubMed:27026396). Regulates osteoblast maturation by controlling TGF-beta bioavailability and calibrating TGF-beta and BMP levels, respectively (By similarity). Negatively regulates osteoclastogenesis by binding and sequestering an osteoclast differentiation and activation factor TNFSF11. This leads to disruption of TNFSF11-induced Ca2+ signaling and impairment of TNFSF11-mediated nuclear translocation and activation of transcription factor NFATC1 which regulates genes important for osteoclast differentiation and function (PubMed:24039232). Mediates cell adhesion via its binding to cell surface receptors integrins ITGAV:ITGB3 and ITGA5:ITGB1 (PubMed:12807887, PubMed:17158881). Binds heparin and this interaction has an important role in the assembly of microfibrils (PubMed:11461921).
Cellular Location Secreted. Note=Fibrillin-1 and Asprosin chains are still linked together during the secretion from cells, but are subsequentely separated by furin (PubMed:24982166). Fibrillin-1: Secreted, extracellular space, extracellular matrix
Research Areas
Citations (0)

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Background

Fibrillins are structural components of 10-12 nm extracellular calcium-binding microfibrils, which occur either in association with elastin or in elastin-free bundles. Fibrillin-1- containing microfibrils provide long-term force bearing structural support. Regulates osteoblast maturation by controlling TGF-beta bioavailability and calibrating TGF-beta and BMP levels, respectively.

References

Pereira L.V.,et al.Hum. Mol. Genet. 2:961-968(1993).
Uyeda T.,et al.J. Hum. Genet. 49:404-407(2004).
Rieder M.J.,et al.Submitted (SEP-2009) to the EMBL/GenBank/DDBJ databases.
Zody M.C.,et al.Nature 440:671-675(2006).
Mural R.J.,et al.Submitted (JUL-2005) to the EMBL/GenBank/DDBJ databases.

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$ 450.00
Cat# ALS14370
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