| 克隆類(lèi)型 ： |
Mouse Monoclonal Antibody |
|
亞 型 ： |
Mouse IgG2b |
|
免 疫 原 ： |
Recombinant human dystrophin fragment. |
|
產(chǎn)品描述 ： |
Dystrophin anchors the extracellular matrix to the cytoskeleton via F-actin. Dystrophin is the ligand for dystroglycan. Dystrophin is a component of the dystrophin-associated glycoprotein complex which accumulates at the neuromuscular junction (NMJ) and at a variety of synapses in the peripheral and central nervous systems and has a structural function in stabilizing the sarcolemma.Defects in DMD are the cause of Duchenne muscular dystrophy (DMD). DMD is the most common form of muscular dystrophy; a sex-linked recessive disorder. It typically presents in boys aged 3 to 7 year as proximal muscle weakness causing waddling gait, toe-walking, lordosis, frequent falls, and difficulty in standing up and climbing up stairs. |
| 適用物種 ： |
H, M, R, Rb |
|
應(yīng)用范圍 ： |
E, WB, IHC |
| 應(yīng)用說(shuō)明 ： |
E: 1:500-1:1,000; WB: 1:100-1:500; IHC: 1:100-1:500 |
| 別 名 ： |
Dystrophin; DMD |
|
形 態(tài) ： |
Each vial contains 0.1 mg IgG in 0.1 ml (1 mg/ml) of PBS pH7.4, 2% BSA with 0.09% sodium azide. Antibody was purified by Protein-G 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/dystrophin-antibody |
| 標(biāo) 記 物 ： |
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產(chǎn)品說(shuō)明 ： |
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