LIFR active protein

LIFR, Recombinant, Mouse (Leukemia Inhibitory Factor Receptor, LIF Receptor, LIF-R, D-factor/LIF Receptor, CD118)

Cat. Num. AAA636029

• Gene Names: LIFR; SWS; SJS2; STWS; CD118; LIF-R
• Reactivity: Mouse
• Purity: Highly Purified; ~95% (SDS-PAGE, reducing conditions)
• Synonyms: LIFR; Recombinant; Mouse (Leukemia Inhibitory Factor Receptor; LIF Receptor; LIF-R; D-factor/LIF Receptor; CD118); LIFR active protein

• Reactivity: Mouse
• Purity/Purification: Highly Purified; ~95% (SDS-PAGE, reducing conditions)
• Form/Format: Supplied as a lyophilized powder from PBS. Reconstitute to 0.5mg/ml with sterile PBS.

• Activity: Measured by its ability to inhibit LIF-dependent proliferation of TF-1 human erythroleukemic cells. The ED50 for this effect is typically 0.8-4ug/ml.
• Endotoxin Level: 1EU/1ug (LAL)
• Preparation and Storage: Lyophilized powder may be stored at -20 degree C. Stable for 12 months at -20 degree C and lower. Reconstituted product is stable for 3 months under sterile conditions at -20 degree C and lower. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Related Product Information for LIFR active protein

Leukemia Inhibitory Factor Receptor alpha (LIF R-alpha), also known as LIFR beta and CD118, is a 190kD type I transmembrane protein in the Interleukin-6 receptor family. Members of this family mediate the biological effects of Cardiotrophin-1, CLC, CNTF, IL-6, IL-11, IL-27, and Oncostatin M. Mature mouse LIF R-alpha consists of a 785aa extracellular domain (ECD) with two cytokine receptor homology domains, one WSxWS motif, and three fibronectin type III repeats, followed by a 25aa transmembrane segment and a 239aa cytoplasmic domain. Within the ECD, mouse LIF R-alpha shares 73% and 90% aa sequence identity with human and rat LIF R-alpha, respectively. Alternative splicing generates a 90kD soluble form of the mouse LIF R-alpha ECD. LIF R-alpha binds the pleiotropic cytokine LIF with low affinity, and the soluble isoform retains LIF-binding activity. Binding affinity is increased by the ligand-induced association of LIF R-alpha with the signal transducing subunit gp130. The LIF R-alpha/gp130 receptor complex also transduces Oncostatin M signals, although LIF R-alpha alone does not interact with Oncostatin M. gp130 associates with different ligand-specific receptors to form signaling receptor complexes for the other IL-6 family ligands. The CNTF receptor is a ternary complex that contains CNTF R-alpha and gp130 as well as LIF R-alpha. LIF R-alpha is widely expressed, and LIF induces the proliferation, differentiation, and activation of cells in many tissues. In particular, LIF R-alpha plays an important role in several aspects of early pregnancy such as blastocyst implantation in the uterus.

Product Categories/Family for LIFR active protein

Molecular Biology; MB-Receptors

NCBI and Uniprot Product Information

• NCBI GI #: 189083786
• NCBI GeneID: 3977
• NCBI Accession #: NP_001121143.1
• NCBI GenBank Nucleotide #: NM_001127671.1
• UniProt Accession #: P42702; Q6LCD9
• Molecular Weight: ~89kD
• NCBI Official Full Name: leukemia inhibitory factor receptor
• NCBI Official Synonym Full Names: leukemia inhibitory factor receptor alpha
• NCBI Official Symbol: LIFR
• NCBI Official Synonym Symbols: SWS; SJS2; STWS; CD118; LIF-R
• NCBI Protein Information: leukemia inhibitory factor receptor; LIF receptor; CD118 antigen
• UniProt Protein Name: Leukemia inhibitory factor receptor
• Protein Family: Leukemia inhibitory factor receptor
• UniProt Gene Name: LIFR
• UniProt Synonym Gene Names: LIF receptor; LIF-R
• UniProt Entry Name: LIFR_HUMAN

NCBI Description

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 adult 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]

Uniprot Description

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: Receptor, cytokine; Membrane protein, integral

Chromosomal Location of Human Ortholog: 5p13-p12

Cellular Component: integral to plasma membrane; receptor complex

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

Biological Process: cell surface receptor linked signal transduction; response to cytokine stimulus; cytokine and chemokine mediated signaling pathway; leukemia inhibitory factor signaling pathway; positive regulation of cell proliferation

Disease: Stuve-wiedemann Syndrome