SIP1 Pure 4/SIP1 50ug
货号：
611256 基本售价：
3690.0 元 规格：
50ug
产品信息
概述| 货号 | 611256 |
| 描述 | Spinal muscular atrophy (SMA), a commonly fatal autosomal recessive disease, is caused by the degeneration of spinal anterior horn cells. It leads to symmetrical limb and trunk paralysis and muscular atrophy. SMA has been linked to the protein product of the Survival of Motor Neurons (SMN) gene. In greater than 98% of all SMA patients, SMN has been reported to be deleted or mutated. Although its function is unknown, the SMN protein is highly concentrated in novel nuclear structures, termed gems. SIP1 (SMN-interactingprotein1) forms a stable heteromeric complex with SMN and colocalizes with SMN in gems and in the cytoplasm. In SMA, the expression of both proteins is dramatically reduced in motor neurons. Additionally, SMN and SIP1 have been isolated from a 300 kDa protein complex that also contains spliceosomal snRNP proteins. In particular, the SMN-SIP1 complex associates with the spliceosomal snRNAs U1 and U5. Antibodies against the SMN-SIP1 complex interfere with the assembly and nuclear importation of spliceosomal complexes. Thus, it is thought that the SMN-SIP1 complex mediates the formation of spliceosomal snRNPs. However, the exact role of SIP1 in rRNA processing has yet to be determined. |
| 别名 | SMN-Interacting Protein 1 |
| 同种亚型 | IgG1 |
| 反应种属 | Human (QC Testing) |
| 来源宿主 | Mouse |
| 使用方法 | WB, IF |
性能| 供应商 | BD Pharmingen |
| 免疫原 | Human SIP1 aa. 36-156 |
| 存放说明 | The monoclonal antibody was purified from tissue culture supernatant or ascites by affinity chromatography.Store undiluted at -20°C.
|
| 存储溶液 | Aqueous buffered solution containing BSA, glycerol, and ≤0.09% sodium azide. |
| 浓度 | 250 µg/ml |
