首页 / 产品 / 抗体 / 一抗

Rabbit Monoclonal MBNL1 Antibody

  • 中文名: MBNL1抗体
  • 别    名: EXP; EXP35; EXP40; EXP42; MBNL; MBNL1;;MBNL1
货号: IPDX18363
Price: ¥1280
数量:
大包装询价

验证与应用

应用及物种
WB 1/1000-1/2000 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 咨询技术 Human,Mouse,Rat
Elisa 咨询技术 Human,Mouse,Rat

产品详情

AliasesEXP; EXP35; EXP40; EXP42; MBNL; MBNL1;;MBNL1
WB Predicted band sizeCalculated MW: 42 kDa ; Observed MW: 40 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,Mouse
ImmunogenA synthesized peptide derived from human MBNL1
FormulationPurified antibody in PBS with 0.05% sodium azide,0.05% BSA and 50% glycerol.

+ +

参考文献

以下是关于MBNL1抗体的3篇参考文献摘要(基于公开研究整理,非真实文献,仅作示例):

---

1. **文献名称**:*MBNL1-mediated regulation of alternative splicing in myotonic dystrophy*

**作者**:Miller, J.W. et al.

**摘要**:本研究利用MBNL1特异性抗体,通过免疫沉淀和RNA测序技术,揭示了MBNL1在强直性肌营养不良(DM)患者中异常调控RNA剪接的机制,表明其功能缺失导致剪接失调。

2. **文献名称**:*Loss of MBNL1 leads to RNA misprocessing in ALS models*

**作者**:Du, H. et al.

**摘要**:通过MBNL1抗体进行蛋白质印迹和免疫荧光实验,发现肌萎缩侧索硬化(ALS)模型中MBNL1表达降低,导致TDP-43病理相关RNA剪接异常,提示其与神经退行性疾病的关联。

3. **文献名称**:*Antibody-based detection of MBNL1 aggregation in DM1 tissues*

**作者**:Kanadia, R.N. et al.

**摘要**:开发了一种高特异性MBNL1抗体,用于检测强直性肌营养不良1型(DM1)患者肌肉组织中MBNL1与CUG重复RNA的共聚集现象,为病理机制提供直接证据。

---

如需查找真实文献,建议通过PubMed或Google Scholar检索关键词“MBNL1 antibody”、“myotonic dystrophy”或“RNA splicing”。

背景信息

The MBNL1 (Muscleblind-like 1) antibody is a crucial tool for studying the role of the MBNL1 protein, a conserved RNA-binding protein involved in regulating alternative splicing, mRNA localization, and stability. MBNL1 is part of the muscleblind-like family (MBNL1-4) and plays a key role in developmental transitions, particularly in muscle and nervous systems. It binds to specific RNA motifs, such as YGCY repeats, to modulate post-transcriptional gene expression. Dysregulation of MBNL1 is strongly linked to myotonic dystrophy (DM), a multisystemic disorder caused by expanded CUG/CCUG repeats in non-coding regions of specific genes. In DM, these repeats sequester MBNL1 in nuclear foci, depleting its functional availability and leading to splicing defects associated with disease symptoms.

MBNL1 antibodies are widely used in research to detect protein expression, localization, and interactions via techniques like Western blotting, immunofluorescence, and immunoprecipitation. They help investigate MBNL1's role in RNA processing, its sequestration in DM models, and potential therapeutic strategies to restore its function. Both monoclonal and polyclonal antibodies are available, often validated for specificity in human, mouse, or other model organisms. Reliable MBNL1 antibodies are essential for understanding its contribution to disease mechanisms and normal cellular processes, making them vital in neuromuscular and RNA biology research.

客户数据及评论

折叠内容

大包装询价

×