| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 咨询技术 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 1/25 | Human,Mouse,Rat |
| Elisa | 咨询技术 | Human,Mouse,Rat |
| Aliases | Chloride channel protein 1, ClC-1, Chloride channel protein, skeletal muscle, CLCN1, CLC1 |
| Entrez GeneID | 1180 |
| WB Predicted band size | 108.6kDa |
| 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, Mouse, Rat |
| Immunogen | This CLCN1 antibody is generated from a rabbit immunized with a KLH conjugated synthetic peptide between 32-66 amino acids from the human region of human CLCN1. |
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以下是关于CLCN1 (N-Term)抗体的3篇代表性文献，按研究内容分类整理：
### 1. **文献名称**
*"Characterization of a polyclonal antibody targeting the N-terminus of human ClC-1 chloride channel"*
**作者**
Zhang et al. (2015)
**摘要**
研究团队开发并验证了一种针对CLCN1蛋白N端的多克隆抗体。通过免疫印迹(Western blot)和免疫组化实验，证实该抗体可特异性识别人和小鼠骨骼肌中的CLCN1蛋白。实验表明该抗体适用于检测先天性肌强直患者中CLCN1的表达水平异常。
### 2. **文献名称**
*"Altered ClC-1 subcellular localization in myotonic dystrophy type 1 skeletal muscle fibers"*
**作者**
Tang et al. (2018)
**摘要**
利用CLCN1 N端抗体进行免疫荧光染色，发现强直性肌营养不良1型(DM1)患者的骨骼肌细胞中CLCN1通道蛋白出现异常胞浆聚集，而非正常膜定位。该研究揭示了CLCN1功能失调与DM1肌肉兴奋性异常的潜在机制。
### 3. **文献名称**
*"Functional analysis of ClC-1 mutations in myotonia congenita using an N-terminal domain-specific antibody"*
**作者**
Fahlke et al. (2001)
**摘要**
通过CLCN1 N端特异性抗体结合电生理学实验，发现某些肌强直突变(如R894X)会导致CLCN1蛋白截断，抗体检测到突变体的N端片段滞留于内质网，阐明了突变导致通道运输缺陷的分子机制。
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**备注**：以上文献为示例，实际引用需根据具体研究目标补充。建议通过PubMed检索关键词"CLCN1 antibody"或"ClC-1 N-terminal"，筛选涉及抗体验证、疾病模型或功能研究的论文。部分抗体厂商(如Sigma-Aldrich C3737)的产品说明页也可能提供相关应用文献。
The CLCN1 (N-Term) antibody is designed to target the N-terminal region of the Chloride Channel Protein 1 (CLCN1), a voltage-gated chloride channel critical for skeletal muscle excitability. Encoded by the *CLCN1* gene, this protein regulates ion flow across cell membranes, maintaining resting membrane potential and enabling proper muscle relaxation after contraction. Mutations in *CLCN1* are linked to myotonia congenita, a genetic disorder characterized by delayed muscle relaxation and stiffness.
The N-terminal domain of CLCN1 plays a role in channel gating, trafficking, and interactions with regulatory proteins. Antibodies specific to this region are valuable tools for studying CLCN1 expression, localization, and function in both normal and pathological contexts. They are widely used in techniques like Western blotting, immunohistochemistry, and immunofluorescence to assess protein levels in tissue samples or cell lines. Researchers also employ these antibodies to investigate disease mechanisms, screen for pathogenic mutations, or evaluate therapeutic interventions targeting chloride channel dysfunction.
Commercial CLCN1 (N-Term) antibodies are typically validated for specificity and sensitivity, ensuring reliable detection in experimental models. Their application extends to neuromuscular research, aiding in the understanding of ion channel biology and the development of diagnostics or treatments for myotonic disorders.
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