Recombinant人LIFR protein (ab114580)

概述

描述

  • 性质
    Recombinant
  • 来源
    Wheat germ
  • 氨基酸序列
    • Accession
    • 种属
      Human
    • 序列
      QKKGAPHDLKCVTNNLQVWNCSWKAPSGTGRGTDYEVCIENRSRSCYQLE KTSIKIPALSHGDYEITINSLHDFGSSTSKFTLNEQNVSLIPDTPEILNL SADFSTSTLY
    • 分子量
      38 kDa including tags
    • 氨基酸
      45 to 154

技术指标

Our Abpromise guarantee covers the use of ab114580 in the following tested applications.

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

  • 应用

    ELISA

    SDS-PAGE

    Western blot

  • 形式
    Liquid
  • 补充说明
    Protein concentration is above or equal to 0.05 µg/ul. Best use within three months from the date of receipt of this protein.
  • 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
    • CD118
    • CD118 antigen
    • FLJ98106
    • FLJ99923
    • Leukemia inhibitory factor receptor
    • Leukemia inhibitory factor receptor alpha
    • LIF R
    • LIF receptor
    • LIF-R
    • Lifr
    • LIFR_HUMAN
    • SJS2
    • STWS
    • SWS
    see all
  • 功能
    Signal-transducing molecule. May have a common pathway with IL6ST. The soluble form inhibits the biological activity of LIF by blocking its binding to receptors on target cells.
  • 疾病相关
    Defects in LIFR are the cause of Stueve-Wiedemann syndrome (SWS) [MIM:601559]; also knowns as Schwartz-Jampel syndrome type 2 (SJS2). SWS is a severe autosomal recessive condition and belongs to the group of the bent-bone dysplasias. SWS is characterized by bowing of the lower limbs, with internal cortical thickening, wide metaphyses with abnormal trabecular pattern, and camptodactyly. Additional features include feeding and swallowing difficulties, as well as respiratory distress and hyperthermic episodes, which cause death in the first months of life. The rare survivors develop progressive scoliosis, spontaneous fractures, bowing of the lower limbs, with prominent joints and dysautonomia symptoms, including temperature instability, absent corneal and patellar reflexes, and smooth tongue.
    Note=A chromosomal aberration involving LIFR is found in salivary gland pleiomorphic adenomas, the most common benign epithelial tumors of the salivary gland. Translocation t(5;8)(p13;q12) with PLAG1.
  • 序列相似性
    Belongs to the type I cytokine receptor family. Type 2 subfamily.
    Contains 6 fibronectin type-III domains.
  • 结构域
    The WSXWS motif appears to be necessary for proper protein folding and thereby efficient intracellular transport and cell-surface receptor binding.
    The box 1 motif is required for JAK interaction and/or activation.
  • 细胞定位
    Secreted and Cell membrane.
  • Information by UniProt

图片

  • 12.5% SDS-PAGE showing ab114580 at approximately 37.73kDa stained with Coomassie Blue.

文献

ab114580 has not yet been referenced specifically in any publications.

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