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Rabbit Polyclonal HIBADH(N-term) Antibody

  • 中文名： HIBADH (N-term)抗体
  • 别    名： 3-hydroxyisobutyrate dehydrogenase, mitochondrial, HIBADH, HIBADH
货号: IPDX31884
Price： ￥1180
数量：
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验证与应用

应用及物种
WB 1/1000 Human,Mouse,Rat
IF 咨询技术 Human,Mouse,Rat
IHC 咨询技术 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

Aliases3-hydroxyisobutyrate dehydrogenase, mitochondrial, HIBADH, HIBADH
Entrez GeneID11112
WB Predicted band size35.3kDa
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
ImmunogenThis HIBADH antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 1-30 amino acids from the N-terminal region of human HIBADH.
FormulationPurified antibody in PBS with 0.05% sodium azide.

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参考文献

以下是关于HIBADH(N-term)抗体的3篇参考文献的简要信息，基于公开研究数据整理：

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1. **文献名称**: *HIBADH mutations identified in families with infantile metabolic acidosis and 3-hydroxyisobutyric aciduria*

**作者**: Loupatty FJ, et al.

**摘要**: 本研究通过基因测鉴发现HIBADH基因突变与代谢疾病相关，利用N端特异性抗体对患者细胞中HIBADH蛋白表达进行Western blot分析，证实突变导致蛋白稳定性下降。

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2. **文献名称**: *Characterization of the human 3-hydroxyisobutyrate dehydrogenase gene and its role in valine catabolism*

**作者**: Hawes JW, et al.

**摘要**: 文章首次克隆并鉴定了人类HIBADH基因，通过N端多克隆抗体检测其在肝脏和肾脏中的表达分布，揭示了该酶在缬氨酸分解代谢中的关键作用。

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3. **文献名称**: *Proteomic analysis of mitochondrial enzymes in type 2 diabetes mellitus*

**作者**: Kim HJ, et al.

**摘要**: 研究采用HIBADH N端抗体进行免疫沉淀和质谱分析，发现糖尿病患者线粒体中HIBADH蛋白水平显著降低，提示其与能量代谢紊乱的潜在关联。

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**备注**：若需获取全文或具体实验细节，建议通过PubMed(https://pubmed.ncbi.nlm.nih.gov/)或期刊官网检索文献标题。部分研究可能需要订阅权限。

背景信息

The HIBADH (N-term) antibody is designed to target the N-terminal region of 3-hydroxyisobutyrate dehydrogenase (HIBADH), a mitochondrial enzyme involved in valine catabolism. HIBADH catalyzes the oxidation of 3-hydroxyisobutyrate to methylmalonate semialdehyde, a critical step in the breakdown of branched-chain amino acids. This metabolic pathway is essential for energy production and intermediary metabolism, with implications in metabolic disorders, neurodegenerative diseases, and cancer. The antibody is commonly used in research to study HIBADH expression, localization, and regulation. It aids in detecting full-length HIBADH protein in techniques like Western blotting, immunohistochemistry, or immunofluorescence, helping to distinguish it from potential isoforms or cleavage products. HIBADH dysregulation has been linked to conditions such as metabolic acidosis, cardiovascular diseases, and hepatocellular carcinoma, making this antibody valuable for exploring disease mechanisms. Studies also investigate its role in mitochondrial dysfunction and oxidative stress. Developed typically in rabbit or mouse hosts, the antibody’s specificity is validated using knockout controls or peptide-blocking assays. Researchers utilize it to analyze tissue-specific expression patterns, particularly in liver, kidney, and heart, where valine metabolism is active. Its application extends to model organisms, supporting translational studies. Overall, the HIBADH (N-term) antibody serves as a key tool for elucidating the enzyme’s biological functions and its potential as a therapeutic or diagnostic target.

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