产品说明
免疫原
SPG11 antibody was raised against a 15 amino acid peptide of human SPG11.
特点和优势
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.
目标描述
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.
联系
The action of this antibody can be blocked using blocking peptide SBP3500003.
外形
Supplied at approx. 1 mg/mL in phosphate buffered saline containing 0.02% sodium azide.
基本信息
| NACRES | NA.41 |
产品性质
| 生物来源 | rabbit |
| 质量水平 | 100 |
| 偶联物 | unconjugated |
| 抗体形式 | affinity isolated antibody |
| antibody product type | primary antibodies |
| 克隆 | polyclonal |
| 形式 | buffered aqueous solution |
| species reactivity | human, rat, mouse |
| technique(s) | immunohistochemistry: suitable indirect ELISA: suitable western blot: suitable |
| NCBI登记号 | AAI53880 |
| UniProt登记号 | Q96JI7 |
| 运输 | dry ice |
| 储存温度 | −20℃ |
| Gene Information | human ... SPG11(80208) |
安全信息
| 储存分类代码 | 12 - Non Combustible Liquids |
| WGK | WGK 2 |
| 闪点(F) | Not applicable |
| 闪点(C) | Not applicable |
