Rabbit Nesprin 1 Polyclonal Antibody | anti-NESP1 antibody
Nesprin 1 Rabbit pAb
IHC: 1:50-1:200
ICC/IF: 1:50-1:200
FC/FACS: 1:50
NCBI and Uniprot Product Information
NCBI Description
This gene encodes a spectrin repeat containing protein expressed in skeletal and smooth muscle, and peripheral blood lymphocytes, that localizes to the nuclear membrane. Mutations in this gene have been associated with autosomal recessive spinocerebellar ataxia 8, also referred to as autosomal recessive cerebellar ataxia type 1 or recessive ataxia of Beauce. Alternatively spliced transcript variants encoding different isoforms have been described. [provided by RefSeq, Jul 2008]
Uniprot Description
SYNE1: 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. May be 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. May be required for centrosome migration to the apical cell surface during early ciliogenesis. Defects in SYNE1 are the cause of spinocerebellar ataxia autosomal recessive type 8 (SCAR8); 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). 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. Belongs to the nesprin family. 9 isoforms of the human protein are produced by alternative splicing.
Protein type: RNA-binding; Membrane protein, integral; Actin-binding
Chromosomal Location of Human Ortholog: 6q25
Cellular Component: nucleoplasm; Golgi apparatus; nuclear outer membrane; postsynaptic membrane; nuclear membrane; sarcomere; cytoskeleton; cytoplasm; integral to membrane; nuclear envelope; nucleus
Molecular Function: actin filament binding; protein binding; protein homodimerization activity; actin binding; lamin binding
Biological Process: muscle cell differentiation; establishment of nucleus localization; Golgi organization and biogenesis; nuclear organization and biogenesis
Disease: Emery-dreifuss Muscular Dystrophy 4, Autosomal Dominant; Spinocerebellar Ataxia, Autosomal Recessive 8
Research Articles on NESP1
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Product Notes
The NESP1 syne1 (Catalog #AAA8543688) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The Nesprin 1 Rabbit pAb reacts with Human, Mouse, Rat and may cross-react with other species as described in the data sheet. AAA Biotech's Nesprin 1 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), Immunohistochemistry (IHC), Immunocytochemistry (ICC), Immunofluorescence (IF), Flow Cytometry (FC/FACS). WB: 1:500-1:2000 IHC: 1:50-1:200 ICC/IF: 1:50-1:200 FC/FACS: 1:50. Researchers should empirically determine the suitability of the NESP1 syne1 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "Nesprin 1, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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