FITC标记的核孔蛋白GLE1抗体
产品名称： FITC标记的核孔蛋白GLE1抗体
英文名称： Anti-GLE1/FITC
产品编号： HZ-13371R-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
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Rabbit Anti-GLE1/FITC Conjugated antibody
FITC标记的核孔蛋白GLE1抗体
| 英文名称 | Anti-GLE1/FITC |
| 中文名称 | FITC标记的核孔蛋白GLE1抗体 |
| 别 名 | GLE 1; GLE1; GLE1 like protein; GLE1 like RNA export mediator; GLE1 RNA export mediator homolog; GLE1 RNA export mediator like (yeast); GLE1-like protein; GLE1_HUMAN; GLE1L; hGLE1; LCCS 1; LCCS; LCCS1; Nucleoporin GLE1. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 染色质和核信号 信号转导 转运蛋白 表观遗传学 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep, |
| 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 | 80kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human GLE1 |
| 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: Protein transport across the nucleus is a selective, multi-step process involving several cytoplasmic factors that mediate protein passage through the nuclear pore complex (NPC). Gle1, also known as GLE1L, is a 698 amino acid protein that localizes to both the nucleus and the cytoplasm and belongs to the Gle1 family. Expressed as two alternatively spliced isoforms, Gle1 associates with the NPC and is required for the transport of poly(A)-containing mRNAs from the nucleus to the cytoplasm. Defects in the gene encoding Gle1 are the cause of lethal congenital contracture syndrome type 1 (LCCS1) and lethal arthrogryposis with anterior horn cell disease (LAAHD), the former of which is characterized by early fetal hydrops and akinesia, micrognatia, pulmonary hypoplasia, pterygia and prenatal death, while the latter is associated with respiratory failure. Function: Required for the export of mRNAs containing poly(A) tails from the nucleus into the cytoplasm. May be involved in the terminal step of the mRNA transport through the nuclear pore complex (NPC). Subunit: Associated with the NPC, it however may not be a stable component of the NPC complex since it shuttles between the nucleus and the cytoplasm. Interacts with nuclear pore complex proteins NUP155 and NUPL2. Isoform 2 does not interact with NUPL2. Able to form a heterotrimer with NUP155 and NUPL2 in vitro. Subcellular Location: Nucleus. Cytoplasm. Shuttles between the nucleus and the cytoplasm. Shuttling is essential for its mRNA export function and Cytoplasm. Nucleus > nuclear pore complex. Shuttles between the nucleus and the cytoplasm. In the nucleus, isoform 1 localizes to the nuclear pore complex and nuclear envelope. Shuttling is essential for its mRNA export function. DISEASE: Defects in GLE1 are the cause of lethal congenital contracture syndrome type 1 (LCCS1) [MIM:253310]; also known as multiple contracture syndrome type Finnish. LCCS is an autosomal recessive disorder characterized by early fetal hydrops and akinesia, micrognatia, pulmonary hypoplasia, pterygia, multiple joint contractures, specific neuropathology with degeneration of anterior horn neurons and extreme skeletal muscle atrophy. LCCS1 leads to prenatal death. Defects in GLE1 are the cause of lethal arthrogryposis with anterior horn cell disease (LAAHD) [MIM:611890]. LAAHD is characterized by fetal akinesia, arthrogryposis and motor neuron loss. LAADH fetus often survive delivery, but die early as a result of respiratory failure. Neuropathological findings resemble those of LCCS1, but are less severe. Similarity: Belongs to the GLE1 family. Database links: Entrez Gene: 2733 Human Entrez Gene: 74412 Mouse Entrez Gene: 362098 Rat GenBank: BC030012 Human Omim: 603371 Human SwissProt: Q53GS7 Human SwissProt: Q8R322 Mouse SwissProt: Q4KLN4 Rat Unigene: 522418 Human Unigene: 275121 Mouse Unigene: 162648 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
蛋白质跨核转运是一个选择性的、多步骤的过程，涉及几个细胞质因子，它们介导蛋白质通过核孔复合物（NPC）。Gle1又称GLE1L，是一种698个氨基酸的蛋白质，定位于细胞核和细胞质，属于Gle1家族。Gle1表达为两种可选择的剪接异构体，与鼻咽癌相关联，并且需要将含有聚(A)的mRNA从细胞核转运到细胞质。Gle1基因缺陷是致死性先天性挛缩综合征1型(LCCS1)和致死性关节病合并前角细胞病(LAAHD)的病因，前者以早期胎儿水肿、运动障碍、小颌症、肺发育不良、翼状胬肉和妊娠为特征。TAL死亡，后者与呼吸衰竭有关。