SAB4500863
Anti-Fibrillin-1 antibody produced in rabbit
affinity isolated antibody
Synonym(e):
FBN, M350 kda glycoprotein component extracellular microfibril, MASS, MFS1, fibrillin-1
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About This Item
Empfohlene Produkte
Biologische Quelle
rabbit
Konjugat
unconjugated
Antikörperform
affinity isolated antibody
Antikörper-Produkttyp
primary antibodies
Klon
polyclonal
Form
buffered aqueous solution
Mol-Gew.
antigen 312 kDa
Speziesreaktivität
human, rat, mouse
Konzentration
~1 mg/mL
Methode(n)
ELISA: 1:40000
immunohistochemistry: 1:50-1:100
NCBI-Hinterlegungsnummer
UniProt-Hinterlegungsnummer
Versandbedingung
wet ice
Lagertemp.
−20°C
Posttranslationale Modifikation Target
unmodified
Angaben zum Gen
human ... FBN1(2200)
Verwandte Kategorien
Allgemeine Beschreibung
Fibrillin-1 is a multidomain cysteine-rich glycoprotein that belongs to the fibrillin protein family. It is encoded by the FBN1 gene mapped to human chromosome 15q21. Fibrillin-1 structure includes 43 calcium-binding epidermal growth factor (cbEGF)–like domains and 78 cysteine-containing TB motifs. The protein is ubiquitously present and is a vital component of elastic fiber–associated microfibrils in connective tissues. Anti-Fibrillin-1 Antibody detects endogenous levels of total Fibrillin-1 protein.
Immunogen
The antiserum was produced against synthesized peptide derived from human Fibrillin-1.
Immunogen Range: 2811-2860
Immunogen Range: 2811-2860
Anwendung
Anti-Fibrillin-1 antibody produced in rabbit has been used in immunohistochemical staining (1:50).
Biochem./physiol. Wirkung
Fibrillin-1 protein plays an important role in the fibrillogenesis in elastic tissues and non-elastic tissues. It acts as an anchoring fiber and induces Smad2 signaling by regulating the bioavailability of endogenous transforming growth factor β1 (TGF-β1). Mutation in the FBN1 gene leads to the development of heritable disorder Marfan syndrome. Mutation of this gene also causes thoracic aortic aneurysms in patients without Marfan syndrome.
Leistungsmerkmale und Vorteile
Evaluate our antibodies with complete peace of mind. If the antibody does not perform in your application, we will issue a full credit or replacement antibody. Learn more.
Physikalische Form
Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
Haftungsausschluss
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
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Lagerklassenschlüssel
10 - Combustible liquids
WGK
nwg
Flammpunkt (°F)
Not applicable
Flammpunkt (°C)
Not applicable
Analysenzertifikate (COA)
Suchen Sie nach Analysenzertifikate (COA), indem Sie die Lot-/Chargennummer des Produkts eingeben. Lot- und Chargennummern sind auf dem Produktetikett hinter den Wörtern ‘Lot’ oder ‘Batch’ (Lot oder Charge) zu finden.
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Chapter 105 - Heritable Diseases of Connective Tissue
Hawaiian Entomological Society. (2016)
Genetic Basis of Aortic Disease
Hawaiian Entomological Society., 91-100 (2018)
Laure Delhon et al.
FASEB journal : official publication of the Federation of American Societies for Experimental Biology, 33(2), 2707-2718 (2018-10-12)
Mutations in the a disintegrin and metalloproteinase with thrombospondin motif-like 2 ( ADAMTSL2) gene are responsible for the autosomal recessive form of geleophysic dysplasia, which is characterized by short stature, short extremities, and skeletal abnormalities. However, the exact function of
H C Dietz et al.
Human molecular genetics, 4 Spec No, 1799-1809 (1995-01-01)
The extracellular microfibril, 10-14 nm in diameter, performs a number of functions, including serving as the scaffolding for deposition of tropoelastin to form elastic fibers. A variety of proteins compose the structure of microfibrils, the most prominent of which are
D M Milewicz et al.
Circulation, 94(11), 2708-2711 (1996-12-01)
Mutations in the FBN1 gene are the cause of the Marfan syndrome, an autosomal dominant disorder with skeletal, ocular, and cardiovascular complications. Aneurysms or dissections of the ascending thoracic aorta are the major cardiovascular complications of the disorder. We tested
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