推荐产品
生物来源
rabbit
质量水平
偶联物
unconjugated
抗体形式
affinity isolated antibody
抗体产品类型
primary antibodies
克隆
polyclonal
形式
buffered aqueous solution
分子量
antigen 312 kDa
种属反应性
human, rat, mouse
浓度
~1 mg/mL
技术
ELISA: 1:40000
immunohistochemistry: 1:50-1:100
NCBI登记号
UniProt登记号
运输
wet ice
储存温度
−20°C
靶向翻译后修饰
unmodified
基因信息
human ... FBN1(2200)
一般描述
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.
免疫原
The antiserum was produced against synthesized peptide derived from human Fibrillin-1.
Immunogen Range: 2811-2860
Immunogen Range: 2811-2860
应用
Anti-Fibrillin-1 antibody produced in rabbit has been used in immunohistochemical staining (1:50).
生化/生理作用
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.
特点和优势
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.
外形
Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
免责声明
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.
WGK
nwg
闪点(°F)
Not applicable
闪点(°C)
Not applicable
法规信息
新产品
Genetic Basis of Aortic Disease
Hawaiian Entomological Society., 91-100 (2018)
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
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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