Not available outside of the UK & Ireland.
Application
Anti-Fibrillin-1 antibody produced in rabbit has been used in immunohistochemical staining (1:50).
Biochem/physiol Actions
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.
Disclaimer
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.
Features and Benefits
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General description
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
Physical form
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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