Product nameAnti-Lamin B Receptor/LBR antibody
See all Lamin B Receptor/LBR primary antibodies
DescriptionRabbit polyclonal to Lamin B Receptor/LBR
Tested applicationsSuitable for: WB, IP, ICC/IFmore details
Species reactivityReacts with: Mouse, Human
Recombinant fragment corresponding to Mouse Lamin B Receptor/LBR aa 1-81.
- HeLa cells; HeLa cell crude extract.
This product was previously labelled as Lamin B Receptor
Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
Storage bufferPreservative: 0.05% Sodium azide
Constituents: 49% PBS, 50% Glycerol
Ammonium sulphate - trace.
Concentration information loading...
PurityImmunogen affinity purified
Purification notesab122919 is affinity purified using recombinant immunogen.
- Pathways and Processes
- Metabolic signaling pathways
- Lipid and lipoprotein metabolism
- Lipid metabolism
Our Abpromise guarantee covers the use of ab122919 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||Use a concentration of 0.2 - 1 µg/ml. Predicted molecular weight: 71 kDa.|
|IP||Use at an assay dependent dilution.|
|ICC/IF||Use at an assay dependent dilution.|
FunctionAnchors the lamina and the heterochromatin to the inner nuclear membrane.
Involvement in diseaseDefects in LBR are a cause of Pelger-Huet anomaly (PHA) [MIM:169400]. PHA is an autosomal dominant inherited abnormality of neutrophils, characterized by reduced nuclear segmentation and an apparently looser chromatin structure. Heterozygotes show hypolobulated neutrophil nuclei with coarse chromatin. Presumed homozygous individuals have ovoid neutrophil nuclei, as well as varying degrees of developmental delay, epilepsy, and skeletal abnormalities.
Defects in LBR are the cause of hydrops-ectopic calcification-moth-eaten skeletal dysplasia (HEM) [MIM:215140]; also known as Greenberg skeletal dysplasia. HEM is a rare autosomal recessive chondrodystrophy characterized by early in utero lethality and, therefore, considered to be nonviable. Affected fetuses typically present with fetal hydrops, short-limbed dwarfism, and a marked disorganization of chondro-osseous calcification and may present with polydactyly and additional nonskeletal malformations.
Defects in LBR may be a cause of Reynolds syndrome (REYNS) [MIM:613471]. It is a syndrome specifically associating limited cutaneous systemic sclerosis and primary biliray cirrhosis. It is characterized by liver disease, telangiectasia, abrupt onset of digital paleness or cyanosis in response to cold exposure or stress (Raynaud phenomenon), and variable features of scleroderma. The liver disease is characterized by pruritis, jaundice, hepatomegaly, increased serum alkaline phosphatase and positive serum mitochondrial autoantibodies, all consistent with primary biliary cirrhosis.
Sequence similaritiesBelongs to the ERG4/ERG24 family.
modificationsPhosphorylated by CDK1 protein kinase in mitosis when the inner nuclear membrane breaks down into vesicles that dissociate from the lamina and the chromatin. It is phosphorylated by different protein kinases in interphase when the membrane is associated with these structures. Phosphorylation of LBR and HP1 proteins may be responsible for some of the alterations in chromatin organization and nuclear structure which occur at various times during the cell cycle.
Cellular localizationNucleus inner membrane.
- Information by UniProt
- DHCR 14B antibody
- DHCR14B antibody
- Integral nuclear envelope inner membrane protein antibody
This product has been referenced in:
- Pappas SS et al. TorsinA dysfunction causes persistent neuronal nuclear pore defects. Hum Mol Genet 27:407-420 (2018). Read more (PubMed: 29186574) »
- Tajik A et al. Transcription upregulation via force-induced direct stretching of chromatin. Nat Mater 15:1287-1296 (2016). Read more (PubMed: 27548707) »