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由来生物
rabbit
品質水準
結合体
unconjugated
抗体製品の状態
affinity isolated antibody
抗体製品タイプ
primary antibodies
クローン
polyclonal
フォーム
buffered aqueous solution
分子量
antigen 312 kDa
交差性
human, rat, mouse
濃度
~1 mg/mL
テクニック
ELISA: 1:40000
immunohistochemistry: 1:50-1:100
NCBIアクセッション番号
UniProtアクセッション番号
輸送温度
wet ice
保管温度
−20°C
ターゲットの翻訳後修飾
unmodified
遺伝子情報
human ... FBN1(2200)
詳細
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
アプリケーション
Anti-Fibrillin-1 antibody produced in rabbit has been used in immunohistochemical staining (1:50).
生物化学的／生理学的作用
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.
特徴および利点
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.
物理的形状
ウサギIgGのPBS溶液（Mg2+およびCa2+を含まず）、pH 7.4、150 mM NaCl、0.02％ アジ化ナトリウム、50％ グリセロール
免責事項
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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保管分類コード
10 - Combustible liquids
WGK
nwg
引火点(°F)
Not applicable
引火点(℃)
Not applicable
最新バージョンのいずれかを選択してください：
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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
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
Genetic Basis of Aortic Disease
Hawaiian Entomological Society., 91-100 (2018)
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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