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Rabbit Polyclonal ACADS Antibody

  • 中文名: ACADS抗体
  • 别    名: SCAD; ACAD3
货号: IPDX07745
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 1/1000-1/2000 Human,Mouse,Rat

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参考文献

以下是关于ACADS抗体的3篇参考文献,涵盖其在疾病诊断和功能研究中的应用:

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1. **文献名称**:*Molecular characterization of short-chain acyl-CoA dehydrogenase deficiency: from gene to disease mechanism*

**作者**:Tein, I., et al.

**摘要**:该研究利用ACADS抗体进行Western blot分析,检测了SCADD患者细胞中短链酰基辅酶A脱氢酶的蛋白质水平,揭示了基因突变导致的酶稳定性下降及功能丧失的分子机制。

2. **文献名称**:*Diagnosis and management of mitochondrial fatty acid oxidation disorders*

**作者**:Gregersen, N., et al.

**摘要**:综述中讨论了ACADS抗体在脂肪酸氧化缺陷疾病(如SCADD)诊断中的应用,包括通过免疫分析评估酶表达量及活性,为临床分型提供依据。

3. **文献名称**:*Tissue-specific expression of short-chain acyl-CoA dehydrogenase in humans: Insights from antibody-based profiling*

**作者**:Nasser, M., et al.

**摘要**:通过免疫组化技术结合ACADS抗体,系统分析了该酶在人类不同组织(如肝脏、肌肉)中的分布特点,揭示了其代谢功能的组织特异性差异。

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以上文献均涉及ACADS抗体在疾病机制研究、诊断技术开发及蛋白质表达分析中的关键作用。如需具体年份或期刊细节,可进一步补充检索。

背景信息

ACADS (short-chain acyl-CoA dehydrogenase) antibody is primarily used in research and diagnostics to detect deficiencies in the SCAD enzyme, a key mitochondrial protein involved in fatty acid β-oxidation. ACADS catalyzes the initial step of short-chain fatty acid metabolism, converting butyryl-CoA to crotonyl-CoA. Mutations in the *ACADS* gene can lead to SCAD deficiency (SCADD), an autosomal recessive disorder characterized by impaired energy production and metabolic crises, though symptoms vary widely, from asymptomatic to severe neurological and muscular manifestations.

ACADS antibodies are crucial tools in Western blotting, immunohistochemistry, or ELISA to assess enzyme expression levels, aiding in the confirmation of SCADD diagnoses. They help identify reduced or absent ACADS protein in patient samples, complementing genetic testing. Additionally, these antibodies are employed in research to study mitochondrial dysfunction, lipid metabolism disorders, and potential links to broader conditions like epilepsy or metabolic syndrome. Some studies also explore autoantibodies against ACADS in autoimmune contexts, though this remains less characterized. Commercial ACADS antibodies are typically raised in animal models (e.g., mice, rabbits) using purified recombinant protein fragments. Researchers must validate specificity due to potential cross-reactivity with related dehydrogenases. Overall, ACADS antibodies bridge biochemical insights with clinical applications, enhancing understanding of metabolic diseases and therapeutic development.

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