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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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614185
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| 3D Structure
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ModBase 3D Structure for P35555
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| Host
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Rabbit
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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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The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
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| Form/Format
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Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
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| Concentration
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100ug/100ul (lot specific)
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| Reactivity Note
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Human (Identities = 100%, Positives = 100%); Mouse (Identities = 92%, Positives = 100%); Rat (Identities = 92%, Positives = 100%)
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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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| Pathway
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Adhesion
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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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| Product Categories/Family for anti-FBN1 antibody
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Cancer; Cardiovascular; Cell Biology; Epigenetics & Nuclear Signaling; Developmental Biologys; Immunology; Drug Discovery Products; Metabolism; Neuroscience; Signal Transduction; Stem Cells; Autophagy antibody
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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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| Testing Data 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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TOP
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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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[Similar Products]
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TOP
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| NCBI Official Full Name
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fibrillin-1
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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; 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; fibrillin 15
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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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| UniProt Entry Name
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FBN1_HUMAN
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| NCBI Summary for FBN1
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This gene encodes a member of the fibrillin family. The encoded protein is a large, extracellular matrix glycoprotein that serve as a structural component of 10-12 nm calcium-binding microfibrils. These microfibrils provide force bearing structural support in elastic and nonelastic connective tissue throughout the body. Mutations in this gene are associated with Marfan syndrome, isolated ectopia lentis, autosomal dominant Weill-Marchesani syndrome, MASS syndrome, and Shprintzen-Goldberg craniosynostosis syndrome. [provided by RefSeq, Jul 2008]
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| UniProt Comments for FBN1
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FBN1: a large, extracellular matrix glycoprotein of the fibrillin family that serves as a structural component of 10-12 nm 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 in elastic and nonelastic connective tissue throughout the body. May regulate osteoblast maturation by controlling TGF-beta bioavailability and calibrating TGF-beta and BMP levels, respectively. Defects in this protein are associated with Marfan syndrome, isolated ectopia lentis, autosomal dominant Weill-Marchesani syndrome, MASS syndrome, and Shprintzen-Goldberg craniosynostosis syndrome. Interacts with COL16A1.
Protein type: Extracellular matrix; Secreted; Secreted, signal peptide
Chromosomal Location of Human Ortholog: 15q21.1
Cellular Component: extracellular matrix; proteinaceous extracellular matrix; extracellular space; microfibril; extracellular region; basement membrane
Molecular Function: protein binding; extracellular matrix structural constituent; calcium ion binding
Biological Process: extracellular matrix disassembly; extracellular matrix organization and biogenesis; heart development; metanephros development; skeletal development
Disease: Ectopia Lentis 1, Isolated, Autosomal Dominant; Mass Syndrome; Weill-marchesani Syndrome 2; Stiff Skin Syndrome; Acromicric Dysplasia; Marfan Syndrome; Geleophysic Dysplasia 2
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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. Autosomal dominant type 1 fibrillinopathy was the major cause for lens subluxation in this cohort despite the fact that families were inbred and thus at higher risk for recessive disease.
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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 
