Product Details

Uniprot No.

Target Names

SYNE1

Alternative Names

Nesprin-1 (Enaptin) (KASH domain-containing protein 1) (KASH1) (Myocyte nuclear envelope protein 1) (Myne-1) (Nuclear envelope spectrin repeat protein 1) (Synaptic nuclear envelope protein 1) (Syne-1), SYNE1, C6orf98 KIAA0796 KIAA1262 KIAA1756 MYNE1

Species Reactivity

Human

Immunogen

A synthesized peptide from human SYNE1 protein

Immunogen Species

Homo sapiens (Human)

Conjugate

Non-conjugated

Clonality

Monoclonal

Isotype

Rabbit IgG

Clone No.

9D11

Purification Method

Affinity-chromatography

Concentration

It differs from different batches. Please contact us to confirm it.

Buffer

Rabbit IgG in 10mM phosphate buffered saline , pH 7.4, 150mM sodium chloride, 0.05% BSA, 0.02% sodium azide and 50% glycerol.

Form

Liquid

Tested Applications

ELISA, WB, IHC, IF, FC

Recommended Dilution

Application	Recommended Dilution
WB	1:500-1:2000
IHC	1:50-1:200
IF	1:50-1:200
FC	1:50-1:200

Protocols

ELISA Protocol
Western Blotting(WB) Protocol
Immunohistochemistry (IHC) Protocol
Immunofluorescence (IF) Protocol
Flow Cytometry (FC) Protocol

Troubleshooting and FAQs

Antibody FAQs

Storage

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.

Lead Time

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

Description

SYNE1, also known as Nesprin-1, is a giant nuclear envelope protein that plays a critical role in connecting the nucleoskeleton to the cytoskeleton through the LINC complex. This spectrin repeat-containing protein is essential for nuclear positioning, mechanotransduction, and maintaining nuclear architecture across diverse cell types. Mutations in SYNE1 have been linked to Emery-Dreifuss muscular dystrophy, cerebellar ataxia, and various cardiomyopathies, making it a significant target for researchers investigating nuclear mechanics, muscle biology, and neurodegenerative disease mechanisms.

This recombinant monoclonal antibody, clone 9D11, offers the reproducibility and consistency that demanding research applications require. Generated against a synthetic peptide from human SYNE1 and produced using recombinant technology, this antibody provides sequence-defined specificity with minimal lot-to-lot variation, ensuring your experimental results remain comparable across studies.

Validation across multiple platforms demonstrates versatile performance in human samples. Western blot analysis in HL-60 and K562 whole cell lysates detects a band at approximately 130 kDa, which represents one of the smaller SYNE1 isoforms rather than the full-length protein predicted at over 1000 kDa, reflecting the complex alternative splicing characteristic of this gene. Immunohistochemistry staining has been validated in paraffin-embedded human glioma tissue, while immunofluorescence studies in A549 cells reveal the expected nuclear envelope localization pattern. Flow cytometry analysis in HepG2 cells further confirms reliable detection in fixed and permeabilized cell preparations.

This antibody serves researchers studying nuclear envelope biology, muscular dystrophies, mechanobiology, and cancer cell nuclear mechanics, providing a dependable tool for investigating SYNE1 expression and localization across multiple experimental workflows.

Usage

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

Target Background

Function

Multi-isomeric modular protein which forms a linking network between organelles and the actin cytoskeleton to maintain the subcellular spatial organization. As a component of the LINC (LInker of Nucleoskeleton and Cytoskeleton) complex involved in the connection between the nuclear lamina and the cytoskeleton. The nucleocytoplasmic interactions established by the LINC complex play an important role in the transmission of mechanical forces across the nuclear envelope and in nuclear movement and positioning. May be involved in nucleus-centrosome attachment and nuclear migration in neural progenitors implicating LINC complex association with SUN1/2 and probably association with cytoplasmic dynein-dynactin motor complexes; SYNE1 and SYNE2 may act redundantly. Required for centrosome migration to the apical cell surface during early ciliogenesis. May be involved in nuclear remodeling during sperm head formation in spermatogenenis; a probable SUN3:SYNE1/KASH1 LINC complex may tether spermatid nuclei to posterior cytoskeletal structures such as the manchette.

Gene References into Functions

The functional integrity of lamin and nesprin-1 is thus required to modulate the FHOD1 activity and the inside-out mechanical coupling that tunes the cell internal stiffness to match that of its soft, physiological-like environment. PMID: 28455503
Authors screened 937 BPD samples for genetic variation in SYNE1 exons 14-33, which covers the CPG2 region, using high-resolution melt analysis. Nine patients are compound heterozygotes for variants in SYNE1/CPG2, suggesting that rare coding variants may contribute significantly towards the complex genetic architecture underlying BPD. PMID: 28178086
The frequency of CACNA1C rs10848683 in genetic high-risk individuals was double that in controls. For SYNE1 rs214950, higher frequencies were found in the genetic high-risk group than in controls. Polymorphisms in CACNA1C and SYNE1 could confer a greater risk of developing Schizophrenia and Bipolar Disorder in individuals who are already at high risk because of their family history. PMID: 27620326
three novel rare variants (R8272Q, S8381C and N8406K) in the C-terminus of the SYNE1 gene (nesprin-1) were identified in seven dilated cardiomyopathy patients by mutation screening. Expression of these mutants caused nuclear morphology defects and reduced lamin A/C and SUN2 staining at the Nuclear envelope. PMID: 28398466
We reported a novel mutation in exon 46 on codon 2304 (G2304R) of the SYNE1 gene in a Chinese family with Emery-Dreifuss muscular dystrophy-like features, and 100 healthy individuals did not show such mutation. PMID: 28583108
Nonsense mutation in the ultimate exon of full-length SYNE1 causes congenital onset of muscular weakness with distal arthrogryposis. PMID: 27782104
The results show that nesprin-1-alpha2 is dynamically controlled and may be involved in some process occurring during early myofibre formation, such as re-positioning of nuclei. PMID: 27350129
findings revise the view that SYNE1 ataxia causes mainly a relatively pure cerebellar recessive ataxia and that it is largely limited to Quebec. Instead, complex phenotypes with a wide range of extra-cerebellar neurological and nonneurological dysfunctions are frequent, including in particular motor neuron and brainstem dysfunction. PMID: 27086870
This study demonstrate four novel truncating mutations in SYNE1 in pedigrees from British, Sri Lankan and Turkish origin in patient with cerebellar ataxia. PMID: 27178001
A full length transcript homologous to rat CPG2 exists within human SYNE1. A full length transcript homologous to rat CPG2 exists within human SYNE1. PMID: 26704904
SNP rs79575945 in ESR1 gene is associated with cancers of endometrioid subtype and resulted in the expression of SYNE1. PMID: 26330482
Mechanostimulation mediates nuclear changes in oligodendrocyte progenitor cells via the SYNE1 complex. PMID: 26791211
our data suggest that SYNE1 and FOXE1 are promising markers for colorectal cancer detection. PMID: 25538088
Drosophila melanogaster larval muscles, exhibiting both elastic features contributed by the stretching capacity of MSP300 (nesprin) and rigidity provided by a perinuclear network of microtubules stabilized by Shot (spectraplakin) and EB1. PMID: 26008743
ur results show that SNPs rs9371601 and rs3093664 in the SYNE1 and TNF genes respectively, are associated with menstrual migraine. PMID: 25315199
A report of a family with a SYNE1 gene mutation that seems to cause an "Emery-Dreifuss muscular dystrophy-like" phenotype. PMID: 25091525
a role for Nesprin-1 in the DNA damage response pathway PMID: 24781983
FOXE1 and SYNE1 hypermethylation markers demonstrated significantly increased expression in neoplastic tissue. PMID: 24280874
These data identify p50(Nesp1) as a multi-functional P-body component and microtubule scaffold necessary for RNA granule dynamics and provides evidence for P-body and stress granule micro-heterogeneity. PMID: 24862572
role of CSMD1 and SYNE1 in the etiology of bipolar disorder PMID: 24387768
Two novel truncating mutations were found among the French-Canadian participants, and 2 other novel mutations were found in a patient from France and a patient from Brazil. PMID: 23959263
The findings of this study add to the evidence that association of SYNE1 mutation influences susceptibility to bipolar and unipolar mood disorders. PMID: 22565781
we report 4 novel homozygous SYNE1 mutations in 3 Japanese patients with cerebellar ataxia PMID: 23325900
These results indicate that nesprin-1 knockdown releases the nucleus from the tension of F-actin bound to the nucleus, thereby increasing allowance for deformation before stretching, and that F-actin bound to the nucleus through nesprin-1 causes sustainable force transmission to the nucleus. PMID: 22728879
analysis of nsprin-1 mutations in human and murine cardiomyopathy PMID: 19944109
Nesprin-1 depletion increased the number of focal adhesions and substrate traction while decreasing the speed of cell migration PMID: 20655839
a single nucleotide polymorphism downstream of ESR1, a gene called spectrin repeat containing, nuclear envelope 1, was associated with invasive ovarian cancer risk PMID: 20056644
Vertebrate Nesprin-1 and nesprin-2 proteins are orthologous to Drosophila melanogaster muscle protein MSP-300 PMID: 12408964
The role of Syne-1 in cytokinesis involves an interaction with kinesin II. PMID: 14709720
Enaptin is an element of the nuclear membrane and the actin cytoskeleton. PMID: 15093733
The Nesprin-1 is essential for the interaction with a C-terminal region in SUN1 protein. PMID: 16079285
These findings indicate that GSRP-56 is a small Golgi-localized splice-isoform of the spectrin-repeat-containing Syne1 gene. PMID: 16875688
SYNE1 is the first identified gene responsible for a recessively inherited pure cerebellar ataxia. PMID: 17159980
This study identified a cluster of French-Canadian families with a new recessive ataxia of relatively pure cerebellar type caused by mutations in SYNE1. PMID: 17503513
Loss of Drop1 expression at early stages in carcinomas of the uterus, cervix, kidney, lung, thyroid and pancreas. PMID: 18709643
splice site mutation of SYNE-1 gene found in the family is responsible for arthrogryposis multiplex congenita. PMID: 19542096

Hide All

Involvement in disease

Spinocerebellar ataxia, autosomal recessive, 8 (SCAR8); Emery-Dreifuss muscular dystrophy 4, autosomal dominant (EDMD4)

Subcellular Location

Nucleus outer membrane; Single-pass type IV membrane protein; Cytoplasmic side. Nucleus. Nucleus envelope. Cytoplasm, cytoskeleton. Cytoplasm, myofibril, sarcomere. Note=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. In myoblasts, relocalized from the nuclear envelope to the nucleus and cytoplasm during cell differentiation.; [Isoform GSRP-56]: Golgi apparatus.

Protein Families

Nesprin family

Tissue Specificity

Expressed in HeLa, A431, A172 and HaCaT cells (at protein level). Widely expressed. Highly expressed in skeletal and smooth muscles, heart, spleen, peripheral blood leukocytes, pancreas, cerebellum, stomach, kidney and placenta. Isoform GSRP-56 is predomi

Database Links

HGNC: 17089

OMIM: 608441

KEGG: hsa:23345

STRING: 9606.ENSP00000356224

UniGene: Hs.12967

Message

Product >> Product Name：
Product >> Code：
Name： *
Email： *
Company/Organization：
Phone：
Since emails may get flagged by spam filters occasionally, we recommend leaving your phone number as a precaution. Your information is safe with us.
Question/Comment： *
Your Region： *
I'd like to receive email newsletters from Cusabio

Yes

No
How do you know CUSABIO?

Email

Distributor

Search engine

Advertisement

Others
CAPTCHA verification：
Please enter the characters shown. Click the image to refresh if unclear.

Code	CSB-RA159195A0HU
Size	US$210
	Order now
Image	Western Blot Positive WB detected in: HL-60 whole cell lysate(30µg), K562 whole cell lysate(30µg) All lanes: Nesprin 1 antibody at 1:1000 Secondary Goat polyclonal to rabbit IgG at 1/40000 dilution Predicted band size: 1011 kDa Observed band size: 130 kDa Exposure time：2min IHC image of CSB-RA159195A0HU diluted at 1:100 and staining in paraffin-embedded human glioma cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.05% DAB. Immunofluorescence staining of A549 cell with CSB-RA159195A0HU at 1:50, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L). Overlay Peak curve showing HepG2 cells stained with CSB-RA159195A0HU (red line) at 1:100. The cells were fixed in 4% formaldehyde and permeated by 0.2% TritonX-100 for 10min. Then 10% normal goat serum to block non-specific protein-protein interactions followed by the antibody (1ug/110⁶cells) for 45min at 4℃. The secondary antibody used was FITC-conjugated goat anti-rabbit IgG (H+L) at 1/200 dilution for 35min at 4℃.Control antibody (green line) was Rabbit IgG (1ug/110⁶cells) used under the same conditions. Acquisition of >10,000 events was performed.
Have Questions?	Leave a Message or Start an on-line Chat

SYNE1 Recombinant Monoclonal Antibody

Product Details

Related Products

Customer Reviews and Q&A

Target Background