克隆類型 : |
Rabbit Polyclonal Antibody |
亞 型 : |
Rabbit Ig |
免 疫 原 : |
KLH-conjugated synthetic peptide encompassing a sequence within the N-term region of human fibrillin-1. |
產(chǎn)品描述 : |
Fibrillins are structural components of 10-12 nm extracellular calcium-binding microfibrils, which occur either in association with elastin or in elastin-free bundles. Fibrillin-1-containing microfibrils provide long-term force bearing structural support. Defects in fibrillin-1 are a cause of Marfan syndrome (MFS), an autosomal dominant disorder that affects the skeletal, ocular, and cardiovascular systems. A wide variety of skeletal abnormalities occurs with MFS, including scoliosis, chest wall deformity, tall stature, abnormal joint mobility. |
適用物種 : |
H, M, R |
應(yīng)用范圍 : |
E, WB, IHC |
應(yīng)用說明 : |
E: 1:500-1:1,000; WB: 1:100-1:500; IHC: 1:100-1:500 |
別 名 : |
FBN1; Fibrillin-1; FBN? |
形 態(tài) : |
Each vial contains 0.1 mg IgG in 0.1 ml (1 mg/ml) of PBS pH7.4, 25% glycerol, 1 mg/ml BSA with 0.09% sodium azide. Antibody was purified by Protein-A affinity chromatography. |
保存條件 : |
Store at -20°C. Minimize freeze-thaw cycles. Product is guaranteed one year from the date of shipment. |
產(chǎn)品鏈接 : |
http://www.abbiotec.com/antibodies/fibrillin-1-antibody |
標(biāo) 記 物 : |
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產(chǎn)品說明 : |
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