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Rabbit Anti-alpha Elastin/Tropoelastin/BF350 Conjugated antibody (bs-1756R-BF350)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-1756R-BF350
英文名稱 Rabbit Anti-alpha Elastin/Tropoelastin/BF350 Conjugated antibody
中文名稱 BF350標(biāo)記的α彈性蛋白抗體
別    名 Elastin alpha; Elastin; Elastin isoform a; ELN; ELN_HUMAN; FLJ38671; FLJ43523; Supravalvular aortic stenosis; SVAS; Tropoelastin; WBS; Williams Beuren syndrome; Williams syndrome region; WS.  
規(guī)格價(jià)格 100ul/2980元 購買        大包裝/詢價(jià)
說 明 書 100ul  
研究領(lǐng)域 腫瘤  免疫學(xué)  神經(jīng)生物學(xué)  轉(zhuǎn)錄調(diào)節(jié)因子  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Mouse,  (predicted: Human, Rat, )
產(chǎn)品應(yīng)用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 70kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Elastin
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
產(chǎn)品介紹 background:
Elastin is a major structural protein of tissues such as aorta, which must expand rapidly and recover completely. Elastin chains are cross-linked together into an extensible 3D network. It is a molecular determinant of late arterial morphogenesis, stabilizing arterial structure by regulating proliferation and organization of vascular smooth muscle. Elastin forms a complex with BGN and MFAP2.

Function:
Major structural protein of tissues such as aorta and nuchal ligament, which must expand rapidly and recover completely. Molecular determinant of the late arterial morphogenesis, stabilizing arterial structure by regulating proliferation and organization of vascular smooth muscle.

Subunit:
The polymeric elastin chains are cross-linked together into an extensible 3D network. Forms a ternary complex with BGN and MFAP2. Interacts with MFAP2 via divalent cations (calcium > magnesium > manganese) in a dose-dependent and saturating manner.

Subcellular Location:
Secreted, extracellular space, extracellular matrix. Note=Extracellular matrix of elastic fibers.

Tissue Specificity:
Expressed within the outer myometrial smooth muscle and throughout the arteriolar tree of uterus (at protein level). Also expressed in the large arteries, lung and skin.

DISEASE:
Defects in ELN are the cause of cutis laxa, autosomal dominant, type 1 (ADCL1) . A connective tissue disorder characterized by loose, hyperextensible skin with decreased resilience and elasticity leading to a premature aged appearance. Face, hands, feet, joints, and torso may be differentially affected. Additional variable clinical features are gastrointestinal diverticula, hernia, and genital prolapse. Rare manifestations are pulmonary artery stenosis, aortic aneurysm, bronchiectasis, and emphysema.

Similarity:
Belongs to the elastin family.

Database links:

Entrez Gene: 2006 Human

Entrez Gene: 13717 Mouse

Omim: 130160 Human

SwissProt: P15502 Human

SwissProt: P54320 Mouse

Unigene: 647061 Human



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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