Cell Biology Antibodies 3

Anti-LIFR Antibody (CAB12783)

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SKU:
CAB12783

Description

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Antibody Name:Anti-LIFR Antibody
Antibody SKU:CAB12783
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Mouse
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 500-700 of human LIFR (NP_002301.1).
Application:WB
Recommended Dilution:WB 1:500 - 1:2000
Reactivity:Mouse
Positive Samples:Mouse skeletal muscle
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 500-700 of human LIFR (NP_002301.1).
Purification Method:Affinity purification
Storage Buffer:Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:NPYT LYTF RIRC STET FWKW SKWS NKKQ HLTT EASP SKGP DTWR EWSS DGKN LIIY WKPL PINE ANGK ILSY NVSC SSDE ETQS LSEI PDPQ HKAE IRLD KNDY IISV VAKN SVGS SPPS KIAS MEIP NDDL KIEQ VVGM GKGI LLTW HYDP NMTC DYVI KWCN SSRS EPCL MDWR KVPS NSTE TVIE SDEF RPGI RYNF F
Gene ID:3977
Uniprot:P42702
Cellular Location:Cell membrane, Secreted, Single-pass type I membrane protein
Calculated MW:123kDa
Observed MW:190kDa
Synonyms:LIFR, CD118, LIF-R, SJS2, STWS, SWS
Background: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.
UniProt Protein Function: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.
UniProt Protein Details:

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; growth factor binding; leukemia inhibitory factor receptor activity; oncostatin-M receptor activity

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

Disease: Stuve-wiedemann Syndrome

NCBI Summary: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 Code:P42702
NCBI GenInfo Identifier:1170784
NCBI Gene ID:3977
NCBI Accession:P42702.1
UniProt Secondary Accession:P42702,Q6LCD9,
UniProt Related Accession:P42702
Molecular Weight:
NCBI Full Name:Leukemia inhibitory factor receptor
NCBI 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
UniProt Synonym Protein Names:CD_antigen: CD118
Protein Family:Leukemia inhibitory factor receptor
UniProt Gene Name:LIFR  
UniProt Entry Name:LIFR_HUMAN
Anti-LIFR Antibody (CAB12783)Western blot analysis of extracts of mouse skeletal muscle, using LIFR antibody (CAB12783) at 1:3000 dilution._Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution._Lysates/proteins: 25ug per lane._Blocking buffer: 3% nonfat dry milk in TBST._Detection: ECL Enhanced Kit (RM00021)._Exposure time: 30s.
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Additional Information

Product Type:
Antibody
Reactivity:
Mouse
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Cell Biology
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