Key features and details
- Rabbit polyclonal to Lamin B Receptor/LBR
- Suitable for: IHC-P, WB
- Reacts with: Mouse, Human
- Isotype: IgG
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: IHC-P, WBmore details
Species reactivityReacts with: Mouse, Human
Synthetic peptide within Human Lamin B Receptor/LBR aa 20-70 conjugated to keyhole limpet haemocyanin. The exact sequence is proprietary.
Database link: Q14739
- IHC-P: Mouse embryo tissue; Human lung carcinoma tissue. WB: Jurkat cell lysate
This product was previously labelled as Lamin B Receptor
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In preparation for this, we have started to update the applications & species that this product is Abpromise guaranteed for.
We are also updating the applications & species that this product has been “predicted to work with,” however this information is not covered by our Abpromise guarantee.
Applications & species from publications and Abreviews that have not been tested in our own labs or in those of our suppliers are not covered by the Abpromise guarantee.
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Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
Storage bufferPreservative: 0.09% Sodium azide
Constituents: 1% BSA, 50% Glycerol
Concentration information loading...
PurityProtein A purified
Our Abpromise guarantee covers the use of ab223013 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|IHC-P||1/100 - 1/500.|
|WB||1/1000. Predicted molecular weight: 71 kDa.|
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
Formalin-fixed, paraffin-embedded mouse embryo tissue stained for Lamin B Receptor/LBR with ab223013 at 1/200 dilution in immunohistochemical analysis. DAB staining.
Formalin-fixed, paraffin-embedded human lung carcinoma tissue stained for Lamin B Receptor/LBR with ab223013 at 1/200 dilution in immunohistochemical analysis. DAB staining.
Anti-Lamin B Receptor/LBR antibody (ab223013) at 1/1000 dilution + Jurkat cell lysate
Predicted band size: 71 kDa
ab223013 has not yet been referenced specifically in any publications.