| 克隆类型 ： |
Rabbit Polyclonal Antibody |
|
亚 型 ： |
Rabbit Ig |
|
免 疫 原 ： |
KLH-conjugated synthetic peptide encompassing a sequence within human SPG11. |
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产品描述 ： |
Hereditary spastic paraplegias (HSPs) are genetically and phenotypically heterogeneous disorders. Spastic paraplegia with thinning of the corpus callosum (ARHSP-TCC) is a relatively frequent form of complicated hereditary spastic paraplegia (cHSP) in which mental retardation and muscle stiffness at onset are followed by slowly progressive paraparesis and cognitive deterioration. Mutations of the SPG11 gene encoding the spatacsin protein have been identified as a major cause of HSP-TCC. Spatacsin is a potential transmembrane protein that is phosphorylated upon DNA damage. It is expressed in all structures of the brain, with a high expression in the cerebellum. SPG11 mutations may occur more frequently in familial than sporadic forms of cHSP without TCC. Kjellin syndrome is found to be associated with mutations in not only the SPG15 gene but also SPG11 gene. Recent studies show Parkinsonism may initiate SPG11-linked HSP TCC and that SPG11 may cause juvenile Parkinsonism. |
| 适用物种 ： |
H, M, R |
|
应用范围 ： |
E, WB, IHC |
| 应用说明 ： |
E: 1:500-1:1,000; WB: 1:100-1:500; IHC: 1:100-1:500 |
| 别 名 ： |
SPG11; Spastic paraplegia 11; colorectal carcinoma-associated protein; spatacsin |
|
形 态 ： |
Each vial contains 0.1 ml IgG in PBS pH 7.4 with 0.02% sodium azide. Antibody was purified by immunogen affinity chromatography. |
| 保存条件 ： |
Store at -20°C. Minimize freeze-thaw cycles. Product is guaranteed one year from the date of shipment. |
| 产品链接 ： |
http://www.abbiotec.com/antibodies/spg11-antibody |
| 标 记 物 ： |
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产品说明 ： |
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