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Rabbit Monoclonal HADHA Antibody

  • 中文名： HADHA抗体
  • 别    名： GBP; ECHA; HADH; LCEH; MTPA; LCHAD; TP-ALPHA
货号: IPDX21477
Price： ￥1280
数量：
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验证与应用

应用及物种
WB 1/500-1/1000 Human,Mouse,Rat
IF 1/20 Human,Mouse,Rat
IHC 1/50-1/100 Human,Mouse,Rat
ICC 1/50-1/200 Human,Mouse,Rat
FCM 咨询技术 Human,Mouse,Rat
Elisa 咨询技术 Human,Mouse,Rat

产品详情

AliasesGBP; ECHA; HADH; LCEH; MTPA; LCHAD; TP-ALPHA
Entrez GeneID3030
WB Predicted band sizeCalculated MW: 83 kDa; Observed MW: 78 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
ImmunogenA synthetic peptide of human HADHA
FormulationPurified antibody in TBS with 0.05% sodium azide,0.05%BSA and 50% glycerol.

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参考文献

以下是关于HADHA抗体的3篇参考文献及其摘要内容：

1. **文献名称**: *"Mitochondrial fatty acid oxidation disorders: clinical presentation of long-chain fatty acid oxidation defects before and after newborn screening"*

**作者**: Rinaldo P. et al.

**摘要**: 该研究分析了长链脂肪酸氧化缺陷(如HADHA基因突变)的临床表现，利用HADHA抗体进行蛋白质表达检测，发现其与新生儿猝死综合征及肝功能障碍相关。

2. **文献名称**: *"The role of HADHA in breast cancer progression: a potential therapeutic target"*

**作者**: Smith J.L. et al.

**摘要**: 通过免疫组化(使用HADHA抗体)发现HADHA在乳腺癌组织中表达下调，其低表达与患者生存率降低相关，提示HADHA可能作为肿瘤代谢治疗的靶点。

3. **文献名称**: *"Antibody-based profiling of mitochondrial proteins in human skeletal muscle reveals deficiencies in fatty acid oxidation in type 2 diabetes"*

**作者**: Koves T.R. et al.

**摘要**: 使用HADHA抗体等工具分析2型糖尿病患者骨骼肌线粒体蛋白，发现HADHA表达减少导致脂肪酸氧化能力下降，与胰岛素抵抗相关。

(注：以上文献为示例，实际引用需根据具体研究核实)

背景信息

The HADHA antibody targets the alpha subunit of the mitochondrial trifunctional protein (MTP), a critical enzyme complex in fatty acid β-oxidation. Encoded by the HADHA gene, this subunit harbors both long-chain enoyl-CoA hydratase and long-chain 3-hydroxyacyl-CoA dehydrogenase activities. MTP catalyzes three sequential steps in breaking down long-chain fatty acids, essential for energy production in tissues like the heart, liver, and skeletal muscle. Antibodies against HADHA are widely used in research to study mitochondrial fatty acid metabolism disorders, particularly LCHAD (long-chain 3-hydroxyacyl-CoA dehydrogenase) deficiency, a life-threatening condition linked to HADHA mutations. These antibodies also aid in exploring metabolic dysregulation in diseases such as diabetes, obesity, and certain cancers. In diagnostics, HADHA antibodies help detect protein expression abnormalities in patient samples. Recent studies have extended their use to investigate the role of impaired fatty acid oxidation in neurodegenerative diseases, cardiac hypertrophy, and ischemia-reperfusion injury. Commercial HADHA antibodies are typically validated via techniques like Western blotting, immunofluorescence, and immunohistochemistry. However, cross-reactivity or batch variability can pose challenges, emphasizing the need for rigorous validation in experimental models.

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