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Anti-C16orf57 Polyclonal Antibody 是一种 Rabbit 抗体,靶向 C16orf57。Anti-C16orf57 Polyclonal Antibody 可用于 WB。
Anti-C16orf57 Polyclonal Antibody 是一种 Rabbit 抗体,靶向 C16orf57。Anti-C16orf57 Polyclonal Antibody 可用于 WB。
| 规格 | 价格 | 库存 | 数量 |
|---|---|---|---|
| 50 μL | ¥ 1,170 | 5日内发货 | |
| 100 μL | ¥ 1,970 | 5日内发货 | |
| 200 μL | ¥ 2,780 | 5日内发货 |
| 产品描述 | Anti-C16orf57 Polyclonal Antibody is a Rabbit antibody targeting C16orf57. Anti-C16orf57 Polyclonal Antibody can be used in WB. |
| Ig Type | IgG |
| 反应种属 | Rat (predicted:Human,Mouse,Dog,Pig,Cow,Horse,Rabbit,Sheep) |
| 应用 | WB |
| 推荐剂量 | WB: 1:500-2000 |
| 抗体种类 | Polyclonal |
| 宿主来源 | Rabbit |
| 亚细胞定位 | Nucleus. |
| 构建方式 | Hybridoma Polyclonal Antibody |
| 纯化方式 | Protein A purified |
| 性状 | Liquid |
| 缓冲液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 浓度 | 1mg/ml |
| 研究背景 | Involvement in disease;Defects in C16orf57 are the cause of poikiloderma with neutropenia (PN). PN is a genodermatosis characterized by poikiloderma, pachyonychia and chronic neutropenia. The disorder starts as a papular erythematous rash on the limbs during the first year of life. It gradually spreads centripetally and, as the papular rash resolves, hypo- and hyperpigmentation result, with development of telangiectasias. Another skin manifestation is pachyonychia, but alopecia and leukoplakia are distinctively absent. One of the most important extracutaneous symptoms is an increased susceptibility to infections, mainly affecting the respiratory system, primarily due to a chronic neutropenia and to neutrophil functional defects. Bone marrow abnormalities account for neutropenia and may evolve into myelodysplasia associated with the risk of leukemic transformation. Poikiloderma with neutropenia shows phenotypic overlap with Rothmund-Thomson syndrome. |
| 免疫原 | KLH conjugated synthetic peptide: human C16orf57 |
| 抗原种属 | Human |
| 基因名称 | USB1 |
| 基因ID | |
| 蛋白名称 | U6 snRNA phosphodiesterase |
| Uniprot ID | |
| 功能 | Putative phosphodiesterase responsible for the U6 snRNA 3' end processing. Acts as a ribonuclease (RNase) responsible for trimming the poly(U) tract of the last nucleotides in the pre-U6 snRNA molecule, leading to the formation of mature U6 snRNA 3' end-terminated with a 2',3'-cyclic phosphate. |
| 分子量 | Theoretical: 30 kDa. |
| 储存方式 | Store at 2°C-8°C for 1 month. Store at -20°C or -80°C for 12 months. Avoid repeated freeze-thaw cycles. |
| 运输方式 | Shipping with blue ice. |
| 存储 | store at low temperature | -20°C for 1 year |