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| Product Name
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Fibrillin-1 (FBN1), Antibody
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| Also Known As
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Fibrillin-1 Antibody
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| Product Synonym Names
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MASS; MFS1; OCTD; SGS; WMS; 350 kda glycoprotein component extracellular microfibril; Weill-Marchesani syndrome; fibrillin 1 (Marfan syndrome); fibrillin 15
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| Product Gene Name
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[Similar Products]
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| Research Use Only
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For Research Use Only. Not for use in diagnostic procedures.
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 TOP
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| OMIM
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102370
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| 3D Structure
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ModBase 3D Structure for P35555
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| Species Reactivity
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Human, Mouse, Rat
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| Specificity
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Fibrillin-1 antibody detects endogenous levels of total Fibrillin-1 protein.
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| Purity/Purification
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Affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
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| Form/Format
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In phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
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| Immunogen
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The antiserum was produced against synthesized peptide derived from human Fibrillin-1.
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| Preparation and Storage
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Store at-20 degree C for 1 year
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| Other Notes
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Small volumes of anti-FBN1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
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TOP
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| Applications Tested/Suitable for anti-FBN1 antibody
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Immunohistochemistry (IHC), ELISA (EIA)
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| Application Notes for anti-FBN1 antibody
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IHC: 1:50-1:100
ELISA: 1:40000
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| Immunohistochemistry (IHC) of anti-FBN1 antibody
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Peptide-+ Immunohistochemical analysis of paraffin-embedded human breast carcinoma tissue, using Fibrillin-1 antibody.
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| NCBI/Uniprot data below describe general gene information for FBN1. It may not necessarily be applicable to this product. |
| NCBI GI #
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281485550
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| NCBI GeneID
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2200
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| NCBI Accession #
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NP_000129.3
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[Other Products]
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| NCBI GenBank Nucleotide #
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NM_000138.4
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[Other Products]
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| UniProt Primary Accession #
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P35555
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[Other Products]
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| UniProt Secondary Accession #
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Q15972; Q75N87; B2RUU0; D2JYH6
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[Other Products]
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| UniProt Related Accession #
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P35555
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[Other Products]
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| Molecular Weight
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312,237 Da
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TOP
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| NCBI Official Full Name
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fibrillin-1 preproprotein
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| NCBI Official Synonym Full Names
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fibrillin 1
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| NCBI Official Symbol
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FBN1
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[Similar Products]
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| NCBI Official Synonym Symbols
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FBN; SGS; WMS; MASS; MFLS; MFS1; OCTD; SSKS; WMS2; ACMICD; ECTOL1; GPHYSD2
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[Similar Products]
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| NCBI Protein Information
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fibrillin-1
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| UniProt Protein Name
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Fibrillin-1
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| Protein Family
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Fibrillin
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| UniProt Gene Name
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FBN1
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[Similar Products]
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| UniProt Synonym Gene Names
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FBN
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[Similar Products]
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| NCBI Summary for FBN1
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This gene encodes a member of the fibrillin family of proteins. The encoded preproprotein is proteolytically processed to generate two proteins including the extracellular matrix component fibrillin-1 and the protein hormone asprosin. Fibrillin-1 is an extracellular matrix glycoprotein that serves as a structural component of calcium-binding microfibrils. These microfibrils provide force-bearing structural support in elastic and nonelastic connective tissue throughout the body. Asprosin, secreted by white adipose tissue, has been shown to regulate glucose homeostasis. Mutations in this gene are associated with Marfan syndrome and the related MASS phenotype, as well as ectopia lentis syndrome, Weill-Marchesani syndrome, Shprintzen-Goldberg syndrome and neonatal progeroid syndrome. [provided by RefSeq, Apr 2016]
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| UniProt Comments for FBN1
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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 (). 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).
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Product References and Citations for anti-FBN1 antibody
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Ehab El-Hallous, J. Biol. Chem., Mar 2007; 282: 8935-8946. Shazia S. Chaudhry, J. Cell Biol., Jan 2007; 176: 355-367. Kerstin Tiedemann, J. Biol. Chem., Sep 2001; 276: 36035-36042. Liliana Schaefer, Am. J. Pathol., Aug 2004; 165: 383-396.
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| Research Articles on FBN1
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1. The overexpression of miR-133b and silence of FBN1 could inhibit the cell proliferative, migratory and invasive abilities of gastric cancer cells, while the influence of down-regulated miR-133b expression and up-regulated FBN1 expression were quite the contrary.
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| Precautions
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All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
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| Disclaimer
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While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.
It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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| Products associated with anti-FBN1 antibody | Pathways associated with anti-FBN1 antibody |
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| Diseases associated with anti-FBN1 antibody | Organs/Tissues associated with anti-FBN1 antibody |
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Antibody 
