Recombinant
RabMAb

Recombinant Anti-Lamin B Receptor/LBR antibody [E398L] (Alexa Fluor® 647) (ab201349)

Overview

  • Product name

    Anti-Lamin B Receptor/LBR antibody [E398L] (Alexa Fluor® 647)
    See all Lamin B Receptor/LBR primary antibodies
  • Description

    Rabbit monoclonal [E398L] to Lamin B Receptor/LBR (Alexa Fluor® 647)
  • Host species

    Rabbit
  • Conjugation

    Alexa Fluor® 647. Ex: 652nm, Em: 668nm
  • Tested applications

    Suitable for: ICC/IFmore details
  • Species reactivity

    Reacts with: Human
  • Immunogen

    Synthetic peptide within Human Lamin B Receptor/LBR. The exact sequence is proprietary.

  • Positive control

    • ICC/IF: HepG2 cells.
  • General notes

     This product was previously labelled as Lamin B Receptor

     

    Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMab® patents.

    Alexa Fluor® is a registered trademark of Molecular Probes, Inc, a Thermo Fisher Scientific Company. The Alexa Fluor® dye included in this product is provided under an intellectual property license from Life Technologies Corporation. As this product contains the Alexa Fluor® dye, the purchase of this product conveys to the buyer the non-transferable right to use the purchased product and components of the product only in research conducted by the buyer (whether the buyer is an academic or for-profit entity). As this product contains the Alexa Fluor® dye the sale of this product is expressly conditioned on the buyer not using the product or its components, or any materials made using the product or its components, in any activity to generate revenue, which may include, but is not limited to use of the product or its components: in manufacturing; (ii) to provide a service, information, or data in return for payment (iii) for therapeutic, diagnostic or prophylactic purposes; or (iv) for resale, regardless of whether they are sold for use in research. For information on purchasing a license to this product for purposes other than research, contact Life Technologies Corporation, 5781 Van Allen Way, Carlsbad, CA 92008 USA or outlicensing@thermofisher.com.

Properties

Applications

Our Abpromise guarantee covers the use of ab201349 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
ICC/IF 1/100.

This product gave a positive signal in HepG2 cells fixed with 100% methanol (5 min).

Target

  • Function

    Anchors the lamina and the heterochromatin to the inner nuclear membrane.
  • Involvement in disease

    Defects 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 similarities

    Belongs to the ERG4/ERG24 family.
  • Post-translational
    modifications

    Phosphorylated 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 localization

    Nucleus inner membrane.
  • Information by UniProt
  • Database links

  • Alternative names

    • DHCR 14B antibody
    • DHCR14B antibody
    • Integral nuclear envelope inner membrane protein antibody
    • Lamin-B receptor antibody
    • LBR antibody
    • LBR_HUMAN antibody
    • LMN 2R antibody
    • LMN2R antibody
    • MGC9041 antibody
    • PHA antibody
    • PRO0650 antibody
    see all

Images

  • ab201349 staining Lamin B Receptor/LBR in HepG2 cells. The cells were fixed with 100% methanol (5 min), permeabilized with 0.1% Triton X-100 for 5 minutes and then blocked with 1% BSA/10% normal goat serum/0.3M glycine in 0.1%PBS-Tween for 1h. The cells were then incubated overnight at +4°C with ab201349 at a 1/100 dilution (shown in red) and ab195887, Mouse monoclonal to alpha Tubulin (Alexa Fluor® 488), at a 1/250 dilution (shown in green). Nuclear DNA was labelled with DAPI (shown in blue).

    Image was taken with a confocal microscope (Leica-Microsystems, TCS SP8).

References

ab201349 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

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Please note: All products are "FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC PROCEDURES"
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