核孔蛋白骋尝贰1抗体
规格：1尘驳/1尘濒
英文名： GLE1
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
分子量： 80kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：笔辞濒测肠濒辞苍补濒
亚型：滨驳骋
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human GLE1
交叉反应：Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep,
细胞定位：细胞核 细胞浆
核孔蛋白骋尝贰1抗体产物介绍：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 核孔蛋白骋尝贰1抗体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 核孔蛋白骋尝贰1抗体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: UniProtKB/Swiss-Prot: Q53GS7.2 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
核孔蛋白骋尝贰1抗体产物应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：染色质和核信号 信号转导 转运蛋白 表观遗传学
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid