| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/20-1/100 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 1/5000-1/10000 | Human,Mouse,Rat |
| Aliases | DUX4L |
| 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 DUX4 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
+ +
以下是关于DUX4抗体的3篇示例参考文献(注：以下内容为示例虚构，实际文献需通过学术数据库检索确认)：
1. **文献名称**：*Development and Validation of a DUX4-Specific Monoclonal Antibody for FSHD Research*
**作者**：Lemmers, R.J.L.F., et al.
**摘要**：本研究开发了一种高特异性单克隆抗体，用于检测面肩肱型肌营养不良症(FSHD)中DUX4蛋白的表达。通过免疫印迹和免疫组化验证，该抗体在患者肌细胞中显示出低水平DUX4的特异性识别，为FSHD病理机制研究提供了关键工具。
2. **文献名称**：*Comparative Analysis of Commercial DUX4 Antibodies in Skeletal Muscle Studies*
**作者**：Tassin, A., et al.
**摘要**：对比了多种市售DUX4抗体的灵敏度和特异性，发现部分抗体在正常肌肉组织中存在非特异性结合。研究推荐了两种适用于免疫荧光和流式细胞术的抗体，并强调了抗体验证在减少假阳性结果中的重要性。
3. **文献名称**：*DUX4 Expression Dynamics in Myogenic Differentiation Revealed by Novel Antibody Tools*
**作者**：Snider, L., et al.
**摘要**：利用新型兔源多克隆抗体，揭示了DUX4在肌细胞分化过程中的短暂表达模式。研究证实了DUX4在FSHD患者肌管细胞中的异常持续性激活，并提供了抗体在活细胞成像中的应用方案。
(提示：实际文献可通过PubMed或Google Scholar以关键词“DUX4 antibody”、“FSHD DUX4 detection”检索。)
The DUX4 antibody is a critical tool in studying the role of the *DUX4* gene, which encodes a double homeobox transcription factor. Normally silenced in most somatic tissues after early embryonic development, DUX4 becomes aberrantly reactivated in facioscapulohumeral muscular dystrophy (FSHD), a debilitating muscle-wasting disorder. In FSHD, chromatin relaxation due to mutations or epigenetic changes on chromosome 4q35 leads to DUX4 expression in skeletal muscle, triggering cytotoxic gene programs that drive muscle degeneration. DUX4 antibodies are designed to detect the DUX4 protein, aiding in research to understand its pathological mechanisms, regulation, and therapeutic targeting.
These antibodies are used in techniques like Western blotting, immunofluorescence, and chromatin immunoprecipitation (ChIP) to visualize DUX4 localization, expression levels, or DNA-binding activity. However, detecting endogenous DUX4 is challenging due to its low abundance in FSHD patient samples and variable specificity of commercial antibodies. Clones such as E5-5 and 9A12 are widely cited, with E5-5 targeting the N-terminal region and 9A12 recognizing the C-terminal domain. Validation using DUX4-knockout cell lines or competitive peptide blocking is essential to confirm antibody specificity.
DUX4 antibodies also have emerging roles in cancer research, as DUX4 is ectopically expressed in certain malignancies. Despite their utility, variability in antibody performance across studies underscores the need for standardized validation protocols and improved reagents to advance FSHD and oncology research.
×
