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Tags & Cell Markers Subcellular Markers Nucleus Nuclear Envelope
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Recombinant Human Lamin B Receptor/LBR protein (ab132387)

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SDS-PAGE - Recombinant Human Lamin B Receptor/LBR protein (ab132387)

    Key features and details

    • Expression system: Wheat germ
    • Suitable for: SDS-PAGE, ELISA, WB

    Description

    • Product name

      Recombinant Human Lamin B Receptor/LBR protein
      See all Lamin B Receptor/LBR proteins and peptides
    • Expression system

      Wheat germ
    • Accession

      Q14739
    • Protein length

      Full length protein
    • Animal free

      No
    • Nature

      Recombinant
      • Species

        Human
      • Sequence

        MPSRKFADGEVVRGRWPGSSLYYEVEILSHDSTSQLYTVKYKDGTELELK ENDIKPLTSFRQRKGGSTSSSPSRRRGSRSRSRSRSPGRPPKSARRSASA SHQADIKEARREVEVKLTPLILKPFGNSISRYNGEPEHIERNDAPHKNTQ EKFSLSQESSYIATQYSLRPRREEVKLKEIDSKEEKYVAKELAVRTFEVT PIRAKDLEFGGVPGVFLIMFGLPVFLFLLLLMCKQKDPSLLNFPPPLPAL YELWETRVFGVYLLWFLIQVLFYLLPIGKVVEGTPLIDGRRLKYRLNGFY AFILTSAVIGTSLFQGVEFHYVYSHFLQFALAATVFCVVLSVYLYMRSLK APRNDLSPASSGNAVYDFFIGRELNPRIGTFDLKYFCELRPGLIGWVVIN LVMLLAEMKIQDRAVPSLAMILVNSFQLLYVVDALWNEEALLTTMDIIHD GFGFMLAFGDLVWVPFIYSFQAFYLVSHPNEVSWPMASLIIVLKLCGYVI FRGANSQKNAFRKNPSDPKLAHLKTIHTSTGKNLLVSGWWGFVRHPNYLG DLIMALAWSLPCGFNHILPYFYIIYFTMLLVHREARDEYHCKKKYGVAWE KYCQRVPYRIFPYIY
      • Predicted molecular weight

        97 kDa including tags
      • Amino acids

        1 to 615

    Associated products

    • Related Products

      • Anti-Lamin B Receptor/LBR antibody [E398L] (ab32535)

    Specifications

    Our Abpromise guarantee covers the use of ab132387 in the following tested applications.

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

    • Applications

      SDS-PAGE

      ELISA

      Western blot

    • Form

      Liquid
    • Additional notes

      This product was previously labelled as Lamin B Receptor.

       

    • Concentration information loading...

    Preparation and Storage

    • Stability and Storage

      Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

      pH: 8.00
      Constituents: 0.31% Glutathione, 0.79% Tris HCl

    General Info

    • Alternative names

      • DHCR 14B
      • DHCR14B
      • Integral nuclear envelope inner membrane protein
      • Lamin-B receptor
      • LBR
      • LBR_HUMAN
      • LMN 2R
      • LMN2R
      • MGC9041
      • PHA
      • PRO0650
      see all
    • 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.
    • Target information above from: UniProt accession Q14739 The UniProt Consortium
      The Universal Protein Resource (UniProt) in 2010
      Nucleic Acids Res. 38:D142-D148 (2010) .

      Information by UniProt

    Images

    • SDS-PAGE - Recombinant Human Lamin B Receptor/LBR protein (ab132387)
      SDS-PAGE - Recombinant Human Lamin B Receptor/LBR protein (ab132387)
      ab132387 on 12.5% SDS-PAGE stained with Coomassie Blue.

    Protocols

    To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.

    Click here to view the general protocols

    Datasheets and documents

    • Datasheet
  • References (0)

    Publishing research using ab132387? Please let us know so that we can cite the reference in this datasheet.

    ab132387 has not yet been referenced specifically in any publications.

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