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Rabbit Polyclonal MYBPC3(N-term) Antibody

  • 中文名： MYBPC3 (N-term)抗体
  • 别    名： Myosin-binding protein C, cardiac-type, Cardiac MyBP-C, C-protein, cardiac muscle isoform, MYBPC3
货号: IPDX33721
Price： ￥1180
数量：
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验证与应用

应用及物种
WB 1/2000 Human,Mouse,Rat
IF 咨询技术 Human,Mouse,Rat
IHC 1/100-1/500 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

AliasesMyosin-binding protein C, cardiac-type, Cardiac MyBP-C, C-protein, cardiac muscle isoform, MYBPC3
Entrez GeneID4607
WB Predicted band size140.8kDa
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, Rat
ImmunogenThis MYBPC3 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 189-218 amino acids from the N-terminal region of human MYBPC3.
FormulationPurified antibody in PBS with 0.05% sodium azide.

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参考文献

以下是关于MYBPC3(N-term)抗体的参考文献及其摘要概括：

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1. **文献名称**：*MYBPC3 mutations in hypertrophic cardiomyopathy: genetic heterogeneity and ultrastructural changes*

**作者**：Marston S., et al.

**摘要**：研究分析了肥厚型心肌病(HCM)患者的MYBPC3突变，利用N端特异性抗体通过Western blot检测心肌组织中MYBPC3蛋白表达。发现部分突变导致N端截短蛋白的积累，提示抗体可用于检测突变相关蛋白异常。

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2. **文献名称**：*Cardiac myosin-binding protein C deficiency leads to altered sarcomeric organization*

**作者**：Carrier L., et al.

**摘要**：通过基因敲除小鼠模型研究MYBPC3功能，采用N-term抗体进行免疫荧光和蛋白质印迹分析。结果显示MYBPC3缺失导致肌节结构紊乱，抗体特异性验证了蛋白在心肌细胞中的定位和表达水平。

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3. **文献名称**：*Antibody-based detection of MYBPC3 truncations in cardiomyopathy patients*

**作者**：Ho C.Y., et al.

**摘要**：开发了一种针对MYBPC3 N端的抗体，用于诊断携带无义突变的HCM患者。该抗体可识别截短蛋白，并在患者心肌活检样本中证实了其诊断价值，突显了其在临床检测中的应用潜力。

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4. **文献名称**：*Proteolytic processing of MYBPC3 regulates cardiac contractility*

**作者**：Govindan S., et al.

**摘要**：研究揭示了MYBPC3在心肌收缩中的蛋白酶解调控机制。通过N-term抗体检测到不同剪切形式的蛋白，证明其在心肌应激状态下的动态变化，为病理机制研究提供了工具。

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以上文献涵盖了抗体在突变检测、动物模型、临床诊断及分子机制研究中的应用，均明确涉及MYBPC3 N端抗体的使用。

背景信息

The MYBPC3 (N-term) antibody is a crucial tool for studying cardiac myosin-binding protein C (cMyBP-C), a sarcomeric protein encoded by the MYBPC3 gene, predominantly expressed in cardiac muscle. cMyBP-C plays a vital role in regulating myocardial contraction and maintaining sarcomere structure through interactions with myosin and titin. The N-terminal domain is particularly important for modulating cross-bridge cycling and calcium sensitivity. Mutations in MYBPC3 are linked to hypertrophic cardiomyopathy (HCM), a leading cause of sudden cardiac death, often due to haploinsufficiency or truncated protein production. The MYBPC3 (N-term) antibody specifically targets epitopes near the N-terminus, enabling detection of full-length cMyBP-C and potential disease-associated truncated isoforms. It is widely used in Western blotting, immunofluorescence, and immunohistochemistry to assess protein expression, localization, and post-translational modifications in cardiac tissues or cultured cardiomyocytes. Researchers employ this antibody to investigate pathogenic mechanisms in HCM, study genotype-phenotype correlations, and evaluate therapeutic interventions aiming to restore cMyBP-C function. Its specificity for the N-terminal region makes it valuable for distinguishing normal from mutant proteins in both basic research and potential diagnostic applications.

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