FITC标记的脊髓性肌萎缩症蛋白Gemin5抗体
产品名称： FITC标记的脊髓性肌萎缩症蛋白Gemin5抗体
英文名称： Anti-Gemin 5/FITC
产品编号： HZ-20266R-FITC
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： ICC=1:50-200 IF=1:50-200
上海沪震实业有限公司
- 联系人 : 鲍丽雯
- 地址 : 上海市闵行区闵北路88弄1-30号第22幢AQ136室
- 邮编 : 200612
- 所在区域 : 上海
- 电话 : 139****0749 点击查看
- 传真 : 点击查看
- 邮箱 : www.shzbio.net
- 二维码 : 点击查看
Rabbit Anti-Gemin 5/FITC Conjugated antibody
FITC标记的脊髓性肌萎缩症蛋白Gemin5抗体
| 英文名称 | Anti-Gemin 5/FITC |
| 中文名称 | FITC标记的脊髓性肌萎缩症蛋白Gemin5抗体 |
| 别 名 | Gemin5; Gemin-5; gem (nuclear organelle) associated protein 5; Gem-associated protein 5; GEMIN5; GEMI5_HUMAN. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 细胞生物 神经生物学 表观遗传学 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | |
| 产品应用 | ICC=1:50-200 IF=1:50-200 not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 169kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 2mg/1ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Gemin 5 |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 储 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存条件 | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
| 产品介绍 | background: Spinal muscular atrophy (SMA) is an autosomal recessive neurodegenerative disease characterized by loss of motor neurons in the spinal cord (1). SMA is caused by deletion or loss-of-function mutations in the SMN (Survival of Motor Neuron) gene (2). Gemin5, the protein product of human chromosome 5q33.3 (3), associates directly with SMN and is a part of the SMN complex containing Gemin2, Gemin3, Gemin4 and Gemin6 as well as several spliceosomal snRNP proteins (2,4). The SMN complex plays an essential role in spliceosomal snRNP assembly in the cytoplasm and is required for pre-mRNA splicing of the nucleus (2). The SMN complex is found in both the cytoplasm and the nucleus (2). The nuclear form is concentrated in subnuclear bodies called gems (Gemini of the coiled bodies) (2,5,6). Gemin5 interacts with several snRNP core proteins including SmB, SmD1, SmD2, SmD3 and SmE (7). The amino terminal half of Gemin5 contains 13 WD repeat domains and a coiled-coil motif near the C-terminus (7). Function: Gemin 5 is part of a large macromolecular complex localized to both the cytoplasm and the nucleus that plays a role in the cytoplasmic assembly of small nuclear ribonucleoproteins (snRNPs). Other members of this complex include SMN, Gemin 2 (SIP1), Gemin 3 (DDX20), and Gemin 4. Subunit: Part of the core SMN complex that contains SMN1, SIP1/GEMIN2, DDX20/GEMIN3, GEMIN4, GEMIN5, GEMIN6, GEMIN7, GEMIN8 and STRAP/UNRIP. Interacts directly with SMN1, SNRPB, SNRPD1, SNRPD2, SNRPD3 and SNRPE. Subcellular Location: Nucleus, nucleoplasm. Nucleus, gem. Cytoplasm. Note=Found both in the nucleoplasm and in nuclear bodies called gems (Gemini of Cajal bodies) that are often in proximity to Cajal (coiled) bodies. Also found in the cytoplasm. Similarity: Belongs to the WD repeat gemin-5 family. Contains 13 WD repeats. Database links: Entrez Gene: 25929 Human Entrez Gene: 216766 Mouse SwissProt: Q8TEQ6 Human SwissProt: Q3UPH2 Mouse SwissProt: Q8BX17 Mouse Unigene: 483921 Human Unigene: 275349 Mouse Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
脊髓性肌萎缩（SMA）是一种常染色体隐性神经退行性疾病，其特征是脊髓内运动神经元丢失（1）。SMA是由运动神经元存活基因（2）的缺失或功能缺失突变引起的。Gemin5是人类染色体5q33.3(3)的蛋白质产物，与SMN直接相关，是包含Gemin2、Gemin3、Gemin4和Gemin6以及若干剪接体snRNP蛋白(2，4)的SMN复合物的一部分。SMN复合物在细胞质中剪接体snRNP组装中起重要作用，并且是核(2)前mRNA剪接所必需的。SMN复合体存在于细胞质和细胞核中（2）。核形式集中于亚核体，称为宝石（双子座的盘绕体）（2，5，6）。Gemin5与几种snRNP核心蛋白相互作用，包括SmB、SmD1、SmD2、SmD3和SmE(7)。Gemin5的氨基端半含有13个WD重复结构域和一个靠近C末端（7）的线圈结构。