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anti-FBN1 antibody :: Fibrillin-1 Antibody

Scan QR to view Datasheet Catalog #    MBS853519
Immunohistochemistry (IHC)
Unit / Price
0.1 mg  /  $215 +1 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

Fibrillin-1 (FBN1), Antibody

 Also Known As   

Fibrillin-1 Antibody

 Product Synonym Names    MASS; MFS1; OCTD; SGS; WMS; 350 kda glycoprotein component extracellular microfibril; Weill-Marchesani syndrome; fibrillin 1 (Marfan syndrome); fibrillin 15
 Product Gene Name   

anti-FBN1 antibody

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 OMIM    102370
 3D Structure    ModBase 3D Structure for P35555
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 Species Reactivity    Human, Mouse, Rat
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 Specificity    Fibrillin-1 antibody detects endogenous levels of total Fibrillin-1 protein.
 Purity/Purification    Affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
 Form/Format    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    The antiserum was produced against synthesized peptide derived from human Fibrillin-1.
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 Preparation and Storage    Store at-20 degree C for 1 year
 Other Notes    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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 Applications Tested/Suitable for anti-FBN1 antibody   

Immunohistochemistry (IHC), ELISA (EIA)

 Application Notes for anti-FBN1 antibody    IHC: 1:50-1:100
ELISA: 1:40000
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 Immunohistochemistry (IHC) of anti-FBN1 antibody    Peptide-+ Immunohistochemical analysis of paraffin-embedded human breast carcinoma tissue, using Fibrillin-1 antibody.
anti-FBN1 antibody Immunohistochemistry (IHC) (IHC) image
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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 #    281485550
 NCBI GeneID    2200
 NCBI Accession #    NP_000129.3 [Other Products]
 NCBI GenBank Nucleotide #    NM_000138.4 [Other Products]
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 UniProt Primary Accession #    P35555 [Other Products]
 UniProt Secondary Accession #    Q15972; Q75N87; B2RUU0; D2JYH6 [Other Products]
 UniProt Related Accession #    P35555 [Other Products]
 Molecular Weight    312,237 Da
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 NCBI Official Full Name    fibrillin-1 preproprotein
 NCBI Official Synonym Full Names    fibrillin 1
 NCBI Official Symbol    FBN1 [Similar Products]
 NCBI Official Synonym Symbols   
FBN; SGS; WMS; MASS; MFLS; MFS1; OCTD; SSKS; WMS2; ACMICD; ECTOL1; GPHYSD2
[Similar Products]
 NCBI Protein Information    fibrillin-1
 UniProt Protein Name    Fibrillin-1
 Protein Family    Fibrillin
 UniProt Gene Name    FBN1 [Similar Products]
 UniProt Synonym Gene Names    FBN [Similar Products]
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 NCBI Summary for FBN1    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    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

   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    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    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.
 Disclaimer    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 antibodyPathways associated with anti-FBN1 antibody
 Reference Product  PubMed Publications
 FBN2 antibody  >31 publications with FBN1 and FBN2
 LTBP1 antibody  >3 publications with FBN1 and LTBP1
 DCN antibody  >3 publications with FBN1 and DCN
 ELN antibody  >2 publications with FBN1 and ELN
 FBLN2 antibody  >2 publications with FBN1 and FBLN2
 VCAN antibody  >1 publications with FBN1 and VCAN
 MYOC antibody  >1 publications with FBN1 and MYOC
 MFAP5 antibody  >1 publications with FBN1 and MFAP5
 MFAP2 antibody  >1 publications with FBN1 and MFAP2
 CALR antibody  >1 publications with FBN1 and CALR
 Products by Pathway  Pathway Diagram
 Elastic Fibre Formation Pathway antibodies  Elastic Fibre Formation Pathway Diagram
 Extracellular Matrix Organization Pathway antibodies  Extracellular Matrix Organization Pathway Diagram
 Integrin Cell Surface Interactions Pathway antibodies  Integrin Cell Surface Interactions Pathway Diagram
 Signal Transduction Pathway antibodies  Signal Transduction Pathway Diagram
Diseases associated with anti-FBN1 antibodyOrgans/Tissues associated with anti-FBN1 antibody
 Disease Name  Pubmed Publications
 Marfan Syndrome Antibodies  >403 publications with FBN1 and Marfan Syndrome
 Cardiovascular Diseases Antibodies  >363 publications with FBN1 and Cardiovascular Diseases
 Heart Diseases Antibodies  >346 publications with FBN1 and Heart Diseases
 Ectopia Lentis Antibodies  >76 publications with FBN1 and Ectopia Lentis
 Aortic Aneurysm, Thoracic Antibodies  >46 publications with FBN1 and Aortic Aneurysm, Thoracic
 Arachnodactyly Antibodies  >31 publications with FBN1 and Arachnodactyly
 Aortic Rupture Antibodies  >18 publications with FBN1 and Aortic Rupture
 Neoplasms Antibodies  >13 publications with FBN1 and Neoplasms
 Weill-Marchesani Syndrome Antibodies  >11 publications with FBN1 and Weill-Marchesani Syndrome
 ECTOPIA LENTIS 1, ISOLATED, AUTOSOMAL DOMINANT Antibodies  >10 publications with FBN1 and ECTOPIA LENTIS 1, ISOLATED, AUTOSOMAL DOMINANT
 Organ/Tissue Name  Pubmed Publications
 Connective Tissue Antibodies  >187 publications with FBN1 and Connective Tissue
 Vascular Antibodies  >73 publications with FBN1 and Vascular
 Heart Antibodies  >52 publications with FBN1 and Heart
 Skin Antibodies  >52 publications with FBN1 and Skin
 Muscle Antibodies  >36 publications with FBN1 and Muscle
 Eye Antibodies  >34 publications with FBN1 and Eye
 Bone Antibodies  >32 publications with FBN1 and Bone
 Lung Antibodies  >16 publications with FBN1 and Lung
 Embryonic Tissue Antibodies  >12 publications with FBN1 and Embryonic Tissue
 Kidney Antibodies  >9 publications with FBN1 and Kidney
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