|Application ||WB, IHC-P, ICC, E, IP|
|Calculated MW||312237 Da|
|Dilution||IHC-P (10 µg/ml),|
|Other Names||FBN1, ACMICD, Fibrillin 1, Fibrillin-1, FBN, ECTOL1, GPHYSD2, MASS, MFS1, OCTD, SSKS, WMS2, Fibrillin 1 (Marfan syndrome), Fibrillin 15, SGS, WMS|
|Target/Specificity||Recognizes human Fibrillin-1. Epitope mapping studies identify the binding site of this antibody to amino-terminal end of the molecule, between amino acid residues 45 and 450. The antibody is reactive with human, chicken, and bovine Fibrillin-1.|
|Reconstitution & Storage||0.02 M phosphate buffer, pH 7.6, 0.25 M sodium chloride, 0.1% sodium azide. +4°C. Store undiluted.|
|Precautions||FBN1 / Fibrillin 1 Antibody is for research use only and not for use in diagnostic or therapeutic procedures.|
|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|
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Provided below are standard protocols that you may find useful for product applications.
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.
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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