Rabbit Polyclonal to Human CD118 / LIF Receptor Alpha; Human CD118 / LIF Receptor Alpha; CD118 antigen; LIF receptor; SJS2; STWS; Sws; LIF-R

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit

Human CD118 / LIFR

Immunoaffinity purified

PBS, 0.09% sodium azide.

Maintain refrigerated at 2-8 degree C for up to 6 months.
For long term storage store at -20 degree C.

Small volumes of anti-CD118 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

LIFR Antibody, CD118 Antibody, CD118 antigen Antibody, LIF receptor Antibody, SJS2 Antibody, STWS Antibody, Sws Antibody, LIF-R Antibody Description: CD118 / LIF Receptor Alpha is a protein that belongs to the type I cytokine receptor family. This protein combines with a high-affinity converter subunit, gp130, to form a receptor complex that mediates the action of the leukemia inhibitory factor, a polyfunctional cytokine that is involved in cellular differentiation, proliferation and survival in the ***** and the embryo. Mutations in this gene cause Schwartz-Jampel syndrome type 2, a disease belonging to the group of the bent-bone dysplasias.

Immunohistochemistry (IHC) Paraffin, Western Blot (WB)

IHC-P (1:100)
WB (1:1000)

Human Colon, Submucosal Plexus: Formalin-Fixed, Paraffin-Embedded (FFPE)

Human Adrenal: Formalin-Fixed, Paraffin-Embedded (FFPE)

Western blot of lysate from K562 cell line, using LIFR antibody diluted at 1:1000. A goat anti-rabbit IgG H&L (HRP) at 1:10000 dilution was used as the secondary antibody. Lysate at 20 ug.

– Da

leukemia inhibitory factor receptor alpha

leukemia inhibitory factor receptor

Leukemia inhibitory factor receptor

LIF receptor; LIF-R??[Similar Products]

LIFR_HUMAN

This gene encodes a protein that belongs to the type I cytokine receptor family. This protein combines with a high-affinity converter subunit, gp130, to form a receptor complex that mediates the action of the leukemia inhibitory factor, a polyfunctional cytokine that is involved in cellular differentiation, proliferation and survival in the ***** and the embryo. Mutations in this gene cause Schwartz-Jampel syndrome type 2, a disease belonging to the group of the bent-bone dysplasias. A translocation that involves the promoter of this gene, t(5;8)(p13;q12) with the pleiomorphic adenoma gene 1, is associated with salivary gland pleiomorphic adenoma, a common type of benign epithelial tumor of the salivary gland. Multiple splice variants encoding the same protein have been found for this gene. [provided by RefSeq, Jul 2008]

LIFR: 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. Defects in LIFR are the cause of Stueve-Wiedemann syndrome (SWS); 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. 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. Belongs to the type I cytokine receptor family. Type 2 subfamily. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Membrane protein, integral; Receptor, cytokine

Chromosomal Location of Human Ortholog: 5p13-p12

Cellular Component: integral to plasma membrane; plasma membrane; receptor complex

Molecular Function: ciliary neurotrophic factor receptor activity; ciliary neurotrophic factor receptor binding; cytokine binding; growth factor binding; leukemia inhibitory factor receptor activity; oncostatin-M receptor activity; protein heterodimerization activity

Biological Process: cell surface receptor linked signal transduction; cytokine and chemokine mediated signaling pathway; leukemia inhibitory factor signaling pathway; neurite morphogenesis; organ regeneration; positive regulation of cell proliferation; response to cytokine stimulus; response to organic cyclic substance

Disease: Stuve-wiedemann Syndrome

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