| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/25-1/100 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 1/1000-1/2000 | Human,Mouse,Rat |
| Aliases | RSS; CFTD; SELN; MDRS1; RSMD1; SEPN1 |
| WB Predicted band size | 66 kDa |
| Host/Isotype | Rabbit IgG |
| Antibody Type | Primary antibody |
| Storage | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| Species Reactivity | Human |
| Immunogen | Synthetic peptide of human SELENON |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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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。
**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.
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