Recombinant人GLE1 protein (ab114682)

概述

描述

  • 性质Recombinant
  • 来源Wheat germ
  • 氨基酸序列
    • AccessionQ53GS7
    • 种属Human
    • 序列RMKGTEGLRLWQEEQERKVQALSEMASEQLKRFDEWKELKQHKEFQDLRE VMEKSSREALGHQEKLKAEHRHRAKILNLKLREAEQQRVKQAEQERLRKE E
    • 分子量37 kDa including tags
    • 氨基酸140 to 240

技术指标

Our Abpromise guarantee covers the use of ab114682 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • 应用

    Western blot

    SDS-PAGE

    ELISA

  • 形式Liquid
  • 补充说明Protein concentration is above or equal to 0.05 mg/ml.
    Best used within three months from the date of receipt.
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.3% Glutathione, 0.79% Tris HCl

常规信息

  • 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
    • LCCS 1
    • LCCS1
    • Nucleoporin GLE1
    see all
  • 功能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).
  • 疾病相关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.
  • 序列相似性Belongs to the GLE1 family.
  • 细胞定位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.
  • Information by UniProt

Recombinant Human GLE1 protein 图像

  • ab114682 analysed on a 12.5% SDS-PAGE gel stained with Coomassie Blue.

Recombinant Human GLE1 protein (ab114682)参考文献

ab114682 has not yet been referenced specifically in any publications.

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