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SELENON抗体

Rabbit Polyclonal SELENON Antibody
询价 20μl 起订
50μl 起订
100μl 起订
湖北 更新日期：2025-05-15

湖北瑞和宁生物科技有限公司

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电话：027-19371278702拨打
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邮箱：19384116026@163.com

产品详情:

中文名称：
SELENON抗体
英文名称：
Rabbit Polyclonal SELENON Antibody
品牌：
艾普蒂
产地：
武汉
保存条件：
Stored at -20°C for 6472 year. Avoid repeated freeze / thaw cycles.
产品类别：
抗体
重组：
否
应用：
WB: IHC-P: IHC-F: ICC/IF: IP:FC:ChIP: ELISA
种属反应性：
Human,Mouse,Rat
宿主：
Rabbit
偶联物：
无
靶点：
SELENON

验证与应用

应用及物种
WB咨询技术 Human,Mouse,Rat
IF咨询技术 Human,Mouse,Rat
IHC1/25-1/100 Human,Mouse,Rat
ICC技术咨询 Human,Mouse,Rat
FCM咨询技术 Human,Mouse,Rat
Elisa1/1000-1/2000 Human,Mouse,Rat
   

产品详情

AliasesRSS; CFTD; SELN; MDRS1; RSMD1; SEPN1
WB Predicted band size66 kDa
Host/IsotypeRabbit IgG
Antibody TypePrimary antibody
StorageStore at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.
Species ReactivityHuman
ImmunogenSynthetic peptide of human SELENON
FormulationPurified antibody in PBS with 0.05% sodium azide and 50% glycerol.

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       Gel: 8%SDS-PAGE, Lysate: 40 μg, Lane: A172 cells, Primary antibody: P12147(SELENON Antibody) at dilution 1/200, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 10 minutes    


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       The image is immunohistochemistry of paraffin-embedded Human thyroid cancer tissue using P12147(SELENON Antibody) at dilution 1/20. (Original magnification: ×200)    


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       The image is immunohistochemistry of paraffin-embedded Human brain tissue using P12147(SELENON Antibody) at dilution 1/20. (Original magnification: ×200)    


           

参考文献

以下是关于SELENON(SEPN1)抗体的3篇代表性文献，涵盖其在不同研究中的应用及发现：

1. **文献名称**：*Mutations of the Selenoprotein N Gene in Patients with Rigid Spine Muscular Dystrophy*

**作者**：Moghadaszadeh, P. et al.

**摘要**：该研究通过免疫组化技术结合SELENON抗体，发现SEPN1基因突变导致患者骨骼肌中硒蛋白N表达缺失，证实其与先天性脊柱僵硬综合征的关联性。

2. **文献名称**：*SEPN1-Related Myopathy: Functional Consequences of Novel Mutations in Selenoprotein N*

**作者**：Allamand, V. et al.

**摘要**：利用SELENON抗体进行Western blot分析，发现特定错义突变(如R128X)导致蛋白截短，破坏内质网定位功能，阐明突变对肌肉细胞氧化应激通路的影响。

3. **文献名称**：*Diagnostic Utility of SELENON Antibody in Congenital Myopathies*

**作者**：Castañeda, M. et al.

**摘要**：评估SELENON抗体在临床诊断中的价值，通过肌肉活检样本的免疫荧光染色，证明其在区分SEPN1相关肌病与其他先天性肌病中的高特异性与敏感性。

**备注**：SELENON抗体相关研究多集中于SEPN1突变导致的先天性肌病(如RSMD1)，上述文献分别从致病机制、功能研究及临床诊断角度展示了其应用。实际文献检索建议通过PubMed或Web of Science平台以“SEPN1 antibody”及“SELENON myopathy”为关键词筛选近5年研究，可获取更前沿数据。

       

背景信息

**Background of SELENON Antibody**

SELENON (Selenoprotein N), encoded by the *SEPN1* gene, is a crucial endoplasmic reticulum (ER)-resident selenoprotein involved in redox homeostasis and calcium regulation. It contains a selenocysteine residue within a conserved thioredoxin-like fold, enabling antioxidant activity critical for protecting cells from oxidative stress. SELENON is highly expressed in skeletal muscle and plays a role in muscle development and maintenance.

Mutations in *SEPN1* are linked to early-onset neuromuscular disorders, collectively termed SEPN1-related myopathies, including rigid spine muscular dystrophy and congenital myopathies. These conditions are characterized by muscle weakness, respiratory insufficiency, and spinal rigidity. SELENON antibodies are essential tools for studying the protein's expression, localization, and function in both healthy and diseased tissues.

Research using SELENON antibodies has advanced understanding of its role in ER stress response, calcium handling, and muscle cell differentiation. These antibodies are widely used in techniques like Western blotting, immunohistochemistry, and immunofluorescence to investigate disease mechanisms, evaluate therapeutic interventions, or validate *SEPN1* gene editing in preclinical models. Their specificity and reliability make them pivotal in bridging molecular insights with clinical applications for selenoprotein-related disorders.

       
SELENON抗体;SELENON;SELENON Antibody;

公司简介

湖北瑞和宁生物科技有限公司研究创新能力， 拥有完整、科学、质量管理体系。并不断致力于技术创新，产品创新和管理创新，赢得了国内外广大用户的信任。

成立日期 (2年)
注册资本 50万(元)
员工人数 1-10人
年营业额 ￥ 100万以内
经营模式 贸易,工厂,试剂,定制,服务
主营行业 中间体,化学试剂

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