Anti-Nesprin 1 antibody (ab58114)

Overview

• Product nameAnti-Nesprin 1 antibodySee all Nesprin 1 primary antibodies ...
• Description
  Mouse monoclonal to Nesprin 1
• Tested applicationsWB more details
• Species reactivity
  Reacts with: Human
  
• Immunogen

  Recombinant fragment, corresponding to amino acids 1561-1671 of Human Nesprin 1

Properties

• FormLiquid
• 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
• IsotypeIgG3
• Light chain typekappa
• Research Areas
  • Tags & Cell Markers
  • Subcellular Markers
  • Nucleus
  • Nuclear Envelope

Applications

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.

Target information above from: UniProt accession Q8NF91 The UniProt Consortium
The Universal Protein Resource (UniProt) in 2010
Nucleic Acids Res. 38:D142-D148 (2010) .

Information by UniProt
• Database links
  • Entrez Gene: 23345 Human
  • Entrez Gene: 23345 Human
  • Omim: 608441 Human
  • SwissProt: Q8NF91 Human
  • SwissProt: Q8NF91 Human
  • Unigene: 12967 Human

• Alternative names
  CPG2 antibodyEnaptin antibodyMyne-1 antibody

  MYNE1 antibodyMyocyte nuclear envelope protein 1 antibodyNesprin-1 antibodyNuclear envelope spectrin repeat protein 1 antibodySCAR8 antibodySynaptic nuclear envelope protein 1 antibodySyne-1 antibodySYNE1 antibodySYNE1_HUMAN antibodySYNE1B antibody

  see all

Anti-Nesprin 1 antibody images

• 

  Western blot - Nesprin 1 antibody (ab58114)
  Western blot against tagged recombinant protein immunogen using ab58114 Nesprin 1 antibody at 1ug/ml. Predicted band size of immunogen is 38 kDa