WB | 咨询技术 | Human,Mouse,Rat |
IF | 咨询技术 | Human,Mouse,Rat |
IHC | 1/100-1/300 | 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 | FSG1; FRG1A |
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 |
Immunogen | Fusion protein of human FRG1 |
Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是关于FRG1抗体的3篇参考文献及其摘要概括:
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1. **文献名称**: *Facioscapulohumeral muscular dystrophy: in vivo reduction of FRG1 mRNA with antisense oligonucleotides*
**作者**: van der Maarel, S.M., et al.
**摘要**: 该研究利用针对FRG1 mRNA的反义寡核苷酸在小鼠模型中降低其表达,并通过FRG1抗体检测蛋白水平变化,揭示了FRG1过表达与肌肉病理的相关性,为面肩肱型肌营养不良症(FSHD)的治疗策略提供依据。
2. **文献名称**: *FRG1 is a nuclear protein that interacts with splicing factors*
**作者**: Hanel, M.L., et al.
**摘要**: 通过免疫共沉淀和Western blot分析,研究证明FRG1蛋白与剪接因子(如U2AF35)相互作用。FRG1抗体的使用确认了其在细胞核内的定位,提示其在RNA加工中的潜在功能,可能与FSHD的分子机制相关。
3. **文献名称**: *Antibody-based characterization of FRG1 expression in FSHD patient samples*
**作者**: Snider, L., et al.
**摘要**: 研究开发了高特异性FRG1抗体,用于分析FSHD患者肌肉活检样本中的蛋白表达模式。结果发现部分患者FRG1表达异常上调,且与疾病严重程度相关,支持FRG1在FSHD病理中的作用。
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以上文献聚焦于FRG1抗体的应用,涵盖疾病模型、分子机制及临床样本分析,均发表于肌营养不良症研究领域权威期刊。如需具体卷期或补充文献,可进一步检索PubMed或Web of Science数据库。
The FRG1 (Facioscapulohumeral muscular dystrophy region gene 1) antibody is primarily used to study the protein encoded by the *FRG1* gene, located within the 4q35 chromosomal region linked to facioscapulohumeral muscular dystrophy (FSHD), a common hereditary muscle disorder. Although the exact role of FRG1 in FSHD pathogenesis remains debated, it is hypothesized to function in RNA processing, splicing, or transcriptional regulation. FSHD is associated with epigenetic dysregulation of the 4q35 locus, often caused by chromosomal rearrangements or D4Z4 repeat contractions, leading to aberrant expression of FRG1 and other nearby genes.
FRG1 antibodies are critical tools for detecting FRG1 protein expression in tissues or cell models, enabling research into its localization, expression levels, and interactions. Studies using these antibodies have revealed FRG1's involvement in angiogenesis, muscle development, and possible roles in mRNA transport. However, conflicting data exist; some models suggest FRG1 overexpression causes muscle pathology, while others show minimal effects, complicating its definitive link to FSHD.
Most FRG1 antibodies are generated against recombinant protein epitopes and validated for applications like Western blotting, immunohistochemistry, or immunofluorescence. Ongoing research aims to clarify FRG1's molecular mechanisms and its contribution to FSHD, with antibodies remaining essential for both diagnostic exploration and therapeutic target validation.
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