Rabbit anti-Human, Mouse LIFR Polyclonal Antibody | anti-LIFR antibody

LIFR Rabbit Polyclonal

Cat. Num. AAA7601006

• Gene Names: LIFR; SWS; SJS2; STWS; CD118; LIF-R
• Reactivity: Human, Mouse
• Applications: ELISA, Western Blot, Immunohistochemistry
• Purity: >=95% as determined by SDS-PAGE; Immunogen Affinity Purified
• Synonyms: LIFR; Polyclonal Antibody; LIFR Rabbit Polyclonal; anti-LIFR antibody

• Host: Rabbit
• Reactivity: Human, Mouse
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: >=95% as determined by SDS-PAGE; Immunogen Affinity Purified
• Form/Format: Liquid
• Sequence Length: 1097

• Applicable Applications for anti-LIFR antibody: ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)
• Immunogen: Leukemia inhibitory factor receptor alpha
• Preparation and Storage: PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20 degree C for 24 months (Avoid repeated freeze / thaw cycles.)

Immunohistochemistry (IHC)

(Immunohistochemistry of paraffin-embedded human skeletal muscle slide using MBS7601006 (LIFR Antibody) at dilution of 1:50)

Western Blot (WB)

(human skeletal muscle tissue were subjected to SDS PAGE followed by western blot with MBS7601006 (LIFR antibody) at dilution of 1:800)

NCBI and Uniprot Product Information

• NCBI GI #: 189083786
• NCBI GeneID: 3977
• NCBI Accession #: NP_001121143.1
• NCBI GenBank Nucleotide #: NM_001127671.1
• Molecular Weight: – Da
• 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
• 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; 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