Overview

  • Product nameAnti-Nesprin 1 antibody
    See all Nesprin 1 primary antibodies
  • Description
    Rabbit polyclonal to Nesprin 1
  • SpecificityThis antibody detects a number of different Nesprin isoforms, including the major 112 kDa Nesprin 1 alpha.
  • Tested applicationsSuitable for: IHC (PFA fixed), WB, ICC/IF, IHC-Frmore details
  • Species reactivity
    Reacts with: Mouse, Human
  • Immunogen

    Synthetic peptide:

    GRSTPNRQKTPRGKC

    , corresponding to amino acids 3212-3226 of Human Nesprin.

  • General notes


    There are two genes encoding members of a new family of type II integral membrane proteins. Both are ubiquitously expressed, and tissue-specific alternative mRNA initiation and splicing generate at least two major isoforms of each protein, with the smaller isoforms being truncated at the N-terminus.These proteins are called Nesprin l and 2 for nuclear envelope spectrin repeat, as they are characterized by the presence of multiple, clustered spectrin repeats, bipartite nuclear localization sequences and a conserved C-terminal, single transmembrane domain. Transient transfection of EGFP-fusion expression constructs demonstrated their localization to the nuclear membrane with a novel C-terminal, TM-domain-containing sequence essential for perinuclear localization. Nesprin l is developmentally regulated in both smooth and skeletal muscle and is relocalized from the nuclear envelope to the nucleus and cytoplasm during C2Cl2 myoblast differentiation. Nesprins may function as "dystrophins of the nucleus" to maintain nuclear organization and structural integrity.

Properties

  • FormLiquid
  • Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
  • Storage bufferPreservative: 0.025% Sodium azide
    Constituent: PBS
  • Concentration information loading...
  • PurityImmunogen affinity purified
  • Purification notesImmunogen affinity purified on a sepharose column followed by concentration via ion exchange chromotography.
  • Primary antibody notesThere are two genes encoding members of a new family of type II integral membrane proteins. Both are ubiquitously expressed, and tissue-specific alternative mRNA initiation and splicing generate at least two major isoforms of each protein, with the smaller isoforms being truncated at the N-terminus.These proteins are called Nesprin l and 2 for nuclear envelope spectrin repeat, as they are characterized by the presence of multiple, clustered spectrin repeats, bipartite nuclear localization sequences and a conserved C-terminal, single transmembrane domain. Transient transfection of EGFP-fusion expression constructs demonstrated their localization to the nuclear membrane with a novel C-terminal, TM-domain-containing sequence essential for perinuclear localization. Nesprin l is developmentally regulated in both smooth and skeletal muscle and is relocalized from the nuclear envelope to the nucleus and cytoplasm during C2Cl2 myoblast differentiation. Nesprins may function as "dystrophins of the nucleus" to maintain nuclear organization and structural integrity.
  • ClonalityPolyclonal
  • IsotypeIgG
  • Research areas

Applications

Our Abpromise guarantee covers the use of ab5250 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
IHC (PFA fixed) Use at an assay dependent concentration. PubMed: 17159980Use at an assay dependent dilution. Perfused with 4% PFA in PBS from (PubMed:17159980).
WB 1/500 - 1/750. Detects a band of approximately 112 kDa.
ICC/IF 1/25 - 1/50. Fix cells with cold methanol: acetone 1: 1 for 10 minutes on ice.
IHC-Fr 1/25 - 1/50. Fix with cold methanol: acetone 1: 1 for 10 minutes on ice.

Target

  • FunctionMulti-isomeric modular protein which forms a linking network between organelles and the actin cytoskeleton to maintain the subcellular spatial organization. Component of SUN-protein-containing multivariate complexes also called LINC complexes which link the nucleoskeleton and cytoskeleton by providing versatile outer nuclear membrane attachment sites for cytoskeletal filaments. Involved in the maintenance of nuclear organization and structural integrity. Connects nuclei to the cytoskeleton by interacting with the nuclear envelope and with F-actin in the cytoplasm. Required for centrosome migration to the apical cell surface during early ciliogenesis.
  • Tissue specificityWidely expressed. Highly expressed in skeletal and smooth muscles, heart, spleen, and peripheral blood leukocytes.
  • Involvement in diseaseDefects in SYNE1 are the cause of spinocerebellar ataxia autosomal recessive type 8 (SCAR8) [MIM:610743]; also known as autosomal recessive cerebellar ataxia type 1 (ARCA1) or recessive ataxia of Beauce. Spinocerebellar ataxia is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCAR8 is an autosomal recessive form.
    Defects in SYNE1 are the cause of Emery-Dreifuss muscular dystrophy type 4 (EDMD4) [MIM:612998]. A degenerative myopathy characterized by weakness and atrophy of muscle without involvement of the nervous system, early contractures of the elbows, Achilles tendons and spine, and cardiomyopathy associated with cardiac conduction defects.
  • Sequence similaritiesBelongs to the nesprin family.
    Contains 1 actin-binding domain.
    Contains 2 CH (calponin-homology) domains.
    Contains 12 HAT repeats.
    Contains 1 KASH domain.
    Contains 31 spectrin repeats.
  • DomainThe KASH domain, which contains a transmembrane domain, mediates the nuclear envelope targeting and is involved in the binding to SUN1 and SUN2 through recognition of their SUN domains.
  • Cellular localizationNucleus outer membrane. Cytoplasm > cytoskeleton. Cytoplasm > myofibril > sarcomere. The largest part of the protein is cytoplasmic, while its C-terminal part is associated with the nuclear envelope, most probably the outer nuclear membrane. In skeletal and smooth muscles, a significant amount is found in the sarcomeres.
  • Information by UniProt
  • Database links
  • Alternative names
    • 8B antibody
    • ARCA1 antibody
    • C6orf98 antibody
    • CPG2 antibody
    • CPG2 full length antibody
    • dJ45H2.2 antibody
    • EDMD4 antibody
    • Enaptin antibody
    • Myne-1 antibody
    • MYNE1 antibody
    • Myocyte nuclear envelope protein 1 antibody
    • Nesp1 antibody
    • Nesprin-1 antibody
    • Nuclear envelope spectrin repeat protein 1 antibody
    • SCAR8 antibody
    • Spectrin repeat containing nuclear envelope 1 antibody
    • Synaptic nuclear envelope protein 1 antibody
    • Synaptic nuclei expressed gene 1 antibody
    • Syne-1 antibody
    • SYNE1 antibody
    • SYNE1_HUMAN antibody
    • SYNE1B antibody
    see all

Anti-Nesprin 1 antibody images

  • ab5250 at a 1/25 dilution staining Nesprin in C2C12 myoblasts by immunofluorescence.
  • Ab5250 at a 1/100 dilution staining Nesprin 1 in human dermal fibroblasts by immunofluorescence.

References for Anti-Nesprin 1 antibody (ab5250)

This product has been referenced in:
  • Imbalzano KM  et al. Nuclear shape changes are induced by knockdown of the SWI/SNF ATPase BRG1 and are independent of cytoskeletal connections. PLoS One 8:e55628 (2013). ICC/IF ; Human . Read more (PubMed: 23405182) »
  • Korkhov VM & Zuber B Direct observation of molecular arrays in the organized smooth endoplasmic reticulum. BMC Cell Biol 10:59 (2009). ICC/IF ; Human . Read more (PubMed: 19703297) »

See all 4 Publications for this product

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I am sorry that this antibody did not perform as stated on the datasheet. If payment has already been made on the original order and you wish to receive a refund, please ask you...

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I am sorry to hear that you failed to get any signal whilst using our ab5250 antibody against Nesprin 1 in IF on mouse samples.

As it is covered under our Abpromise to work in both IF and mouse, and since it has failed to do so, I would be mor...

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Application Immunocytochemistry/ Immunofluorescence
Sample Human Cell (fibroblasts, embryonic stem cells)
Specification fibroblasts, embryonic stem cells
Fixative Paraformaldehyde
Permeabilization Yes - 0.5% Triton x100
Blocking step Serum as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 10% · Temperature: RT°C
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Verified customer

Submitted Aug 19 2010

Thank you for the details that you have provided and for your patience. I have contacted the originator of this antibody for more information but have unfortunately have not heard back yet. I can offer you a replacement vial free of charge or a refund....

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