Anti-Nesprin 1 antibody (ab58114)
- Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
- Storage bufferPreservative: None
PBS, pH 7.2
- Concentration information loading...
- PurityProtein G purified
- Clonality Monoclonal
- Light chain typekappa
- Research Areas
Our Abpromise guarantee covers the use of ab58114 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
This antibody has only been tested in WB against the recombinant fragment used as immunogen. We have no data on the detection of endogenous protein.
Not yet tested in other applications.
Optimal dilutions/concentrations should be determined by the end user.
- 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.
- CPG2 antibody
- Enaptin antibody
- Myne-1 antibody
- MYNE1 antibody
- Myocyte nuclear envelope protein 1 antibody
- Nesprin-1 antibody
- Nuclear envelope spectrin repeat protein 1 antibody
- SCAR8 antibody
- Synaptic nuclear envelope protein 1 antibody
- Syne-1 antibody
- SYNE1 antibody
- SYNE1_HUMAN antibody
- SYNE1B antibody
Anti-Nesprin 1 antibody images
Western blot against tagged recombinant protein immunogen using ab58114 Nesprin 1 antibody at 1ug/ml. Predicted band size of immunogen is 38 kDa
References for Anti-Nesprin 1 antibody (ab58114)
ab58114 has not yet been referenced specifically in any publications.