Overview

Description

  • Nature

    Recombinant
  • Source

    Baculovirus infected insect cells
  • Amino Acid Sequence
    • Accession
    • Species

      Human
    • Molecular weight

      91 kDa including tags
    • Amino acids

      45 to 833
    • Tags

      His tag C-Terminus
    • Additional sequence information

      corresponding to the extracellular domain

Specifications

Our Abpromise guarantee covers the use of ab219221 in the following tested applications.

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

  • Applications

    SDS-PAGE

  • Endotoxin level

    < 1.000 Eu/µg
  • Purity

    > 90 % SDS-PAGE.
    purified by using conventional chromatography techniques.
  • Form

    Liquid
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.

    pH: 7.4
    Constituents: 90% PBS, 10% Glycerol

General Info

  • Alternative names

    • 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
  • Function

    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.
  • Involvement in disease

    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.
  • Sequence similarities

    Belongs to the type I cytokine receptor family. Type 2 subfamily.
    Contains 6 fibronectin type-III domains.
  • Domain

    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.
  • Cellular localization

    Secreted and Cell membrane.
  • Information by UniProt

Images

  • 15% SDS-PAGE (3μg)

References

ab219221 has not yet been referenced specifically in any publications.

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