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Rabbit Polyclonal MMAB Antibody

  • 中文名: MMAB抗体
  • 别    名: ATR; cob; cblB; CFAP23
货号: IPDX10332
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

AliasesATR; cob; cblB; CFAP23
WB Predicted band size27 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
ImmunogenFusion protein of human MMAB
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MMAB(甲基丙二酰-CoA变位酶相关蛋白)抗体相关的文献示例(注:内容为概括性描述,非真实文献):

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1. **文献名称**:*"Development of a Novel Monoclonal Antibody for Detecting MMAB Mutations in Methylmalonic Acidemia"*

**作者**:Li, X. et al.

**摘要**:研究团队开发了一种高特异性的单克隆抗体,用于识别与甲基丙二酰辅酶A变位酶缺乏症(cblB型)相关的MMAB蛋白突变体。该抗体通过ELISA和免疫印迹验证,可区分健康人群与患者的MMAB表达差异,为临床诊断提供新工具。

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2. **文献名称**:*"Structural Insights into MMAB Protein Function via Antibody-Based Epitope Mapping"*

**作者**:Smith, J.R. & Gonzalez, A.

**摘要**:通过制备多克隆抗体并分析其结合表位,本研究揭示了MMAB蛋白的活性位点构象及其与辅酶A的相互作用机制。结果提示特定抗体可阻断MMAB功能,为潜在治疗策略提供依据。

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3. **文献名称**:*"MMAB Autoantibodies as Biomarkers in Neurological Disorders"*

**作者**:Wang, Y. et al.

**摘要**:首次报道了在部分神经退行性疾病患者血清中检测到抗MMAB自身抗体。研究提示这些抗体可能干扰线粒体代谢,并与认知功能障碍相关,为疾病机制研究开辟新方向。

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(注:如需真实文献,建议通过PubMed或Google Scholar检索关键词"MMAB antibody"或"methylmalonic acidemia cblB type"获取。)

背景信息

MMAB antibodies target methylmalonic acidemia type B (MMAB), a rare inherited metabolic disorder caused by mutations in the *MMAB* gene. This gene encodes the mitochondrial enzyme cobalamin adenosyltransferase (MMAB), which converts vitamin B12 (cobalamin) into its active cofactor form, adenosylcobalamin (AdoCbl). AdoCbl is essential for the function of methylmalonyl-CoA mutase (MUT), a key enzyme in propionate metabolism that converts methylmalonyl-CoA to succinyl-CoA. Defects in MMAB disrupt this pathway, leading to toxic accumulation of methylmalonic acid and related metabolites, resulting in life-threatening complications such as metabolic acidosis, neurological impairment, and multi-organ dysfunction.

MMAB antibodies are critical tools in research and diagnostics. They enable detection and quantification of MMAB protein expression in patient samples, aiding in confirming MMA type B diagnoses and differentiating it from other cobalamin metabolism disorders. Additionally, these antibodies support mechanistic studies to explore disease pathogenesis, evaluate therapeutic interventions (e.g., gene therapy, enzyme replacement), and monitor treatment efficacy. Their development underscores the importance of understanding molecular pathways in rare diseases and advancing precision medicine approaches for MMA patients.

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