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SAB4500863

Sigma-Aldrich

Anti-Fibrillin-1 antibody produced in rabbit

affinity isolated antibody

同義詞:

FBN, M350 kda glycoprotein component extracellular microfibril, MASS, MFS1, fibrillin-1

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About This Item

MDL號碼:
分類程式碼代碼:
12352203
NACRES:
NA.41

生物源

rabbit

共軛

unconjugated

抗體表格

affinity isolated antibody

抗體產品種類

primary antibodies

無性繁殖

polyclonal

形狀

buffered aqueous solution

分子量

antigen 312 kDa

物種活性

human, rat, mouse

濃度

~1 mg/mL

技術

ELISA: 1:40000
immunohistochemistry: 1:50-1:100

NCBI登錄號

UniProt登錄號

運輸包裝

wet ice

儲存溫度

−20°C

目標翻譯後修改

unmodified

基因資訊

human ... FBN1(2200)

一般說明

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

應用

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.

特點和優勢

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.

外觀

Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.

免責聲明

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.

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儲存類別代碼

10 - Combustible liquids

水污染物質分類(WGK)

nwg

閃點(°F)

Not applicable

閃點(°C)

Not applicable


分析證明 (COA)

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存取文件庫

Chapter 105 - Heritable Diseases of Connective Tissue
Hawaiian Entomological Society. (2016)
Genetic Basis of Aortic Disease
Hawaiian Entomological Society., 91-100 (2018)
Shazia S Chaudhry et al.
The Journal of cell biology, 176(3), 355-367 (2007-01-24)
We have discovered that fibrillin-1, which forms extracellular microfibrils, can regulate the bioavailability of transforming growth factor (TGF) beta1, a powerful cytokine that modulates cell survival and phenotype. Altered TGFbeta signaling is a major contributor to the pathology of Marfan

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