Overview

  • Product name
    Anti-Nesprin1 / Syne-1 antibody
    See all Nesprin1 / Syne-1 primary antibodies
  • Description
    Rabbit polyclonal to Nesprin1 / Syne-1
  • Host species
    Rabbit
  • Specificity
    This antibody detects a number of different Nesprin isoforms, including the major 112 kDa Nesprin 1 alpha.
  • Tested applications
    Suitable for: IHC (PFA fixed), WB, ICC/IF, IHC-Frmore details
  • Species reactivity
    Reacts with: Mouse, Human
  • Immunogen

    Synthetic peptide corresponding to Human Nesprin1/ Syne-1 aa 3212-3226.
    Sequence:

    GRSTPNRQKTPRGKC

  • 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.

    Previously labelled as Nesprin 1. 

Properties

  • Form
    Liquid
  • Storage instructions
    Shipped 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 buffer
    Preservative: 0.025% Sodium azide
    Constituent: PBS
  • Concentration information loading...
  • Purity
    Immunogen affinity purified
  • Purification notes
    Immunogen affinity purified on a sepharose column followed by concentration via ion exchange chromotography.
  • Primary antibody 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.
  • Clonality
    Polyclonal
  • Isotype
    IgG
  • 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

  • Function
    Multi-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 specificity
    Widely expressed. Highly expressed in skeletal and smooth muscles, heart, spleen, and peripheral blood leukocytes.
  • Involvement in disease
    Defects 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 similarities
    Belongs 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.
  • Domain
    The 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 localization
    Nucleus 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
    • 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

Images

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

References

This product has been referenced in:
  • Garcia-Manteiga JM  et al. REST-Governed Gene Expression Profiling in a Neuronal Cell Model Reveals Novel Direct and Indirect Processes of Repression and Up-Regulation. Front Cell Neurosci 9:438 (2015). Read more (PubMed: 26617488) »
  • 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) »
See all 5 Publications for this product

Customer reviews and Q&As

1-4 of 4 Abreviews or Q&A

Question
Answer

Thank you for your reply.

Your credit note ID is *.


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 your purchasing department to contact our accounting department so that we may gather the correct information needed for the refund. To avoid confusion, please ensure your accounts department is aware of how the credit note is being used.

Our accounting department can be contacted by email at us.credits@abcam.com or by telephone using the information at the Contact Us link in the top right corner of our website. Please refer to the credit note ID in any correspondence with our accounting department.

The credit note ID is for your reference only and does not automatically guarantee the credit.

I hope this experience will not prevent you from purchasing other products from us in the future. Our Scientific Support team is always at your service, should you require further expert advice.

Read More

Question
Answer

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 more than happy to either replace or refund the cost of the antibody.

Please let me know how you would like to continue and I will be happy to help resolve this issue.

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

Abcam user community

Verified customer

Submitted Aug 19 2010

Answer

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. Please let me know which you would prefer.

Read More

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

Sign up