SAB4500863
Anti-Fibrillin-1 antibody produced in rabbit
affinity isolated antibody
别名:
FBN, M350 kda glycoprotein component extracellular microfibril, MASS, MFS1, fibrillin-1
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关于此项目
Conjugate:
unconjugated
Clone:
polyclonal
Application:
ELISA
immunohistochemistry
immunohistochemistry
Species reactivity:
human, rat, mouse
Citations:
6
Technique(s):
ELISA: 1:40000
immunohistochemistry: 1:50-1:100
immunohistochemistry: 1:50-1:100
Uniprot accession no.:
产品名称
Anti-Fibrillin-1 antibody produced in rabbit, affinity isolated antibody
biological source
rabbit
conjugate
unconjugated
antibody form
affinity isolated antibody
antibody product type
primary antibodies
clone
polyclonal
form
buffered aqueous solution
mol wt
antigen 312 kDa
species reactivity
human, rat, mouse
concentration
~1 mg/mL
technique(s)
ELISA: 1:40000
immunohistochemistry: 1:50-1:100
NCBI accession no.
UniProt accession no.
shipped in
wet ice
storage temp.
−20°C
target post-translational modification
unmodified
Quality Level
Gene Information
human ... FBN1(2200)
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
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.
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
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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存储类别
10 - Combustible liquids
wgk
nwg
flash_point_f
Not applicable
flash_point_c
Not applicable
法规信息
新产品
此项目有
Chapter 105 - Heritable Diseases of Connective Tissue
Hawaiian Entomological Society. (2016)
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
Genetic Basis of Aortic Disease
Hawaiian Entomological Society., 91-100 (2018)
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
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
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