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Rabbit Polyclonal MMUT Antibody

  • 中文名: MMUT抗体
  • 别    名: MCM; MUT
货号: IPDX03581
Price: ¥1180
数量:
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验证与应用

应用及物种
WB 咨询技术 Human,Mouse,Rat
IF 咨询技术 Human,Mouse,Rat
IHC 1/50-1/200 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

产品详情

AliasesMCM; MUT
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
ImmunogenFusion protein of human MMUT
FormulationPurified antibody in PBS with 0.05% sodium azide and 50% glycerol.

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参考文献

以下是3篇与MMUT(甲基丙二酰辅酶A变位酶)抗体相关的文献摘要示例(注:文献信息为模拟,实际引用需通过学术数据库核实):

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1. **文献名称**: *"Characterization of MMUT-Specific Antibodies for Immunoblot Analysis in Methylmalonic Acidemia"*

**作者**: Smith J, et al.

**摘要**: 该研究开发并验证了针对人类MMUT蛋白的高特异性多克隆抗体,通过Western blot分析发现MMA患者细胞中MMUT蛋白表达显著降低,为疾病诊断提供了实验依据。

2. **文献名称**: *"Impact of Mutations on MMUT Protein Stability Studied by Immunoprecipitation"*

**作者**: Garcia R, et al.

**摘要**: 利用抗MMUT抗体进行免疫共沉淀实验,发现某些致病突变会导致MMUT酶构象改变并加速降解,揭示了部分MMA患者的分子病理机制。

3. **文献名称**: *"Subcellular Localization of MMUT in Mitochondria Using Confocal Microscopy"*

**作者**: Lee H, et al.

**摘要**: 通过免疫荧光标记结合MMUT抗体,证实MMUT蛋白定位于线粒体基质,并发现特定突变会破坏其线粒体靶向信号,影响酶功能。

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**建议**:实际文献检索可使用PubMed或Google Scholar,以关键词“MMUT antibody”或“methylmalonyl-CoA mutase antibody”筛选,并优先选择近5年内的研究。

背景信息

MMUT antibody targets methylmalonyl-CoA mutase (MCM), a mitochondrial enzyme encoded by the *MMUT* gene, which plays a critical role in catabolizing branched-chain amino acids, odd-chain fatty acids, and cholesterol derivatives. MCM facilitates the isomerization of methylmalonyl-CoA to succinyl-CoA, a key step in the mitochondrial Krebs cycle and energy production. Mutations in *MMUT* are linked to methylmalonic acidemia (MMA), a rare inherited metabolic disorder characterized by the accumulation of methylmalonic acid, leading to life-threatening complications such as metabolic acidosis, neurological impairment, and multi-organ dysfunction. MMUT antibodies are essential tools in research and diagnostics, enabling the detection of MCM protein expression levels, localization, and potential dysfunction in patient samples or experimental models. These antibodies are widely used in techniques like Western blotting, immunohistochemistry, and immunofluorescence to study disease mechanisms, assess gene therapy outcomes, or validate cellular models of MMA. Additionally, they aid in distinguishing MMA subtypes caused by *MMUT* mutations from those arising from defects in other genes (e.g., *MMAA*, *MMAB*), informing targeted therapeutic strategies.

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