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anti-FBN1 antibody :: Rabbit Fibrillin-1 Antibody

Scan QR to view Datasheet Catalog #    MBS853519
Testing Data
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    614185
 3D Structure    ModBase 3D Structure for P35555
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 Host    Rabbit
 Species Reactivity    Human, Mouse, Rat
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 Specificity    Fibrillin-1 antibody detects endogenous levels of total Fibrillin-1 protein.
 Purity/Purification    The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
 Form/Format    Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
 Concentration    100ug/100ul (lot specific)
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 Reactivity Note    Human (Identities = 100%, Positives = 100%); Mouse (Identities = 92%, Positives = 100%); Rat (Identities = 92%, Positives = 100%)
 Immunogen    The antiserum was produced against synthesized peptide derived from human Fibrillin-1.
 Pathway    Adhesion
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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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 Product Categories/Family for anti-FBN1 antibody    Cancer; Cardiovascular; Cell Biology; Epigenetics & Nuclear Signaling; Developmental Biologys; Immunology; Drug Discovery Products; Metabolism; Neuroscience; Signal Transduction; Stem Cells; Autophagy antibody
 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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 Testing Data of anti-FBN1 antibody    Peptide - +
Immunohistochemical analysis of paraffin-embedded human breast carcinoma tissue, using Fibrillin-1 antibody.
anti-FBN1 antibody Testing Data 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 [Similar Products]
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 NCBI Official Full Name    fibrillin-1
 NCBI Official Synonym Full Names    fibrillin 1
 NCBI Official Symbol    FBN1 [Similar Products]
 NCBI Official Synonym Symbols   
FBN; SGS; WMS; MASS; MFS1; OCTD; SSKS; WMS2; ACMICD; ECTOL1; GPHYSD2
[Similar Products]
 NCBI Protein Information    fibrillin-1; fibrillin 15
 UniProt Protein Name    Fibrillin-1
 Protein Family    Fibrillin
 UniProt Gene Name    FBN1 [Similar Products]
 UniProt Synonym Gene Names    FBN [Similar Products]
 UniProt Entry Name    FBN1_HUMAN
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 NCBI Summary for FBN1    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    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

   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. 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    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
 DCN antibody  >4 publications with FBN1 and DCN
 ELN antibody  >4 publications with FBN1 and ELN
 FBLN2 antibody  >2 publications with FBN1 and FBLN2
 ITGB6 antibody  >1 publications with FBN1 and ITGB6
 ITGA5 antibody  >1 publications with FBN1 and ITGA5
 ITGAV antibody  >1 publications with FBN1 and ITGAV
 MFAP5 antibody  >1 publications with FBN1 and MFAP5
 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
Diseases associated with anti-FBN1 antibodyOrgans/Tissues associated with anti-FBN1 antibody
 Disease Name  Pubmed Publications
 Marfan Syndrome Antibodies  >460 publications with FBN1 and Marfan Syndrome
 Cardiovascular Diseases Antibodies  >403 publications with FBN1 and Cardiovascular Diseases
 Heart Diseases Antibodies  >380 publications with FBN1 and Heart Diseases
 Ectopia Lentis Antibodies  >91 publications with FBN1 and Ectopia Lentis
 Aortic Aneurysm, Thoracic Antibodies  >59 publications with FBN1 and Aortic Aneurysm, Thoracic
 Disease Models, Animal Antibodies  >40 publications with FBN1 and Disease Models, Animal
 Arachnodactyly Antibodies  >35 publications with FBN1 and Arachnodactyly
 Aortic Rupture Antibodies  >24 publications with FBN1 and Aortic Rupture
 Nervous System Diseases Antibodies  >19 publications with FBN1 and Nervous System Diseases
 Weill-Marchesani Syndrome Antibodies  >17 publications with FBN1 and Weill-Marchesani Syndrome
 Organ/Tissue Name  Pubmed Publications
 Connective Tissue Antibodies  >213 publications with FBN1 and Connective Tissue
 Vascular Antibodies  >84 publications with FBN1 and Vascular
 Heart Antibodies  >63 publications with FBN1 and Heart
 Skin Antibodies  >62 publications with FBN1 and Skin
 Eye Antibodies  >41 publications with FBN1 and Eye
 Bone Antibodies  >38 publications with FBN1 and Bone
 Muscle Antibodies  >38 publications with FBN1 and Muscle
 Lung Antibodies  >18 publications with FBN1 and Lung
 Embryonic Tissue Antibodies  >14 publications with FBN1 and Embryonic Tissue
 Kidney Antibodies  >10 publications with FBN1 and Kidney
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