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Rabbit Polyclonal HADHA Antibody

  • 中文名: HADHA抗体
  • 别    名: GBP; ECHA; HADH; LCEH; MTPA; LCHAD; TP-ALPHA
货号: IPDX01508
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 1/25-1/100 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

AliasesGBP; ECHA; HADH; LCEH; MTPA; LCHAD; TP-ALPHA
WB Predicted band size83 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, Rat
ImmunogenFusion protein of human HADHA
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HADHA抗体相关的文献摘要(注:文献标题和作者为模拟内容,仅供参考):

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1. **"Mitochondrial Trifunctional Protein Deficiency: Molecular Insights from HADHA Antibody-Based Analysis"**

*Authors: J. Smith et al. (2020)*

摘要:通过HADHA特异性抗体研究线粒体三功能蛋白α亚基(HADHA)在患者成纤维细胞中的表达缺失,揭示其与脂肪酸氧化障碍及婴儿猝死综合征的关联。

2. **"Immunohistochemical Detection of HADHA in Cardiac Tissue: Implications for Metabolic Cardiomyopathy"**

*Authors: L. Chen & R. Tanaka (2018)*

摘要:利用HADHA抗体进行心脏组织免疫组化分析,发现HADHA表达降低与脂肪酸代谢异常相关的心肌病存在直接联系,为临床诊断提供生物标志物。

3. **"CRISPR-Cas9 Editing of HADHA Validated by Western Blot with Novel Polyclonal Antibodies"**

*Authors: K. Park et al. (2021)*

摘要:开发新型HADHA多克隆抗体,验证CRISPR敲除细胞模型中HADHA蛋白的缺失,证实其在研究线粒体功能障碍中的高特异性应用。

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**说明**:以上文献为示例,实际引用需通过PubMed或Google Scholar检索真实文献(关键词:HADHA antibody, mitochondrial trifunctional protein, diagnostic applications)。若需具体文献,建议补充检索条件(如研究领域或疾病类型)。

背景信息

The HADHA antibody is a tool used to detect the hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha (HADHA), a key enzyme in mitochondrial fatty acid β-oxidation. HADHA, along with HADHB (beta subunit), forms the mitochondrial trifunctional protein (MTP), which catalyzes three steps in fatty acid breakdown: long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD), enoyl-CoA hydratase, and thioesterase activities. Mutations in the HADHA gene are linked to inherited metabolic disorders like trifunctional protein deficiency (TFP) and isolated LCHAD deficiency, which cause life-threatening conditions such as cardiomyopathy, hepatic dysfunction, and hypoglycemia. The HADHA antibody is crucial for research and diagnostics, enabling the detection of protein expression levels, localization, and pathogenic variants via techniques like Western blot, immunohistochemistry, or immunofluorescence. It aids in studying metabolic diseases, mitochondrial dysfunction, and cellular energy metabolism pathways. Recent studies also explore its role in cancer, as altered fatty acid metabolism is a hallmark of tumor progression. Commercially available HADHA antibodies are typically validated for specificity and sensitivity, often targeting epitopes within conserved regions of the human HADHA protein. Proper controls are essential to distinguish between full-length HADHA and degradation products or isoforms.

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