首页 / 产品 / 抗体 / 一抗

Rabbit Monoclonal ACADS Antibody

  • 中文名: ACADS抗体
  • 别    名: SCAD; ACAD3
货号: IPDX20998
Price: ¥1280
数量:
大包装询价

验证与应用

应用及物种
WB 咨询技术 Human,Mouse,Rat
IF 1/20 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

AliasesSCAD; ACAD3
Entrez GeneID35
WB Predicted band sizeCalculated MW: 44 kDa; Observed MW: 44 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 ACADS
FormulationPurified antibody in TBS with 0.05% sodium azide,0.05%BSA and 50% glycerol.

+ +

参考文献

以下是关于ACADS(短链酰基辅酶A脱氢酶)抗体的3篇参考文献及其摘要内容概要:

1. **文献名称**: *"Short-chain acyl-CoA dehydrogenase deficiency: clinical and biochemical studies in 45 patients"*

**作者**: Gregersen N, et al.

**摘要**: 该研究分析了45例ACADS基因突变患者的临床和生化特征,利用ACADS抗体进行Western blot检测,发现患者肌肉和成纤维细胞中酶蛋白表达显著降低,揭示了基因型与表型的相关性。

2. **文献名称**: *"Immunohistochemical localization of short-chain acyl-CoA dehydrogenase in human tissues"*

**作者**: Bennett MJ, et al.

**摘要**: 通过免疫组化技术,使用特异性ACADS抗体定位该酶在人类肝脏、心肌和骨骼肌中的分布,发现其在能量需求高的组织中高表达,支持其在脂肪酸代谢中的关键作用。

3. **文献名称**: *"Molecular characterization of ACADS variants in Chinese patients with short-chain acyl-CoA dehydrogenase deficiency"*

**作者**: Wang J, et al.

**摘要**: 研究报道了中国人群中ACADS基因突变谱,结合抗体介导的蛋白质稳定性分析,发现部分错义突变导致酶稳定性下降,为新生儿筛查和诊断提供了分子依据。

背景信息

The ACADS (acyl-CoA dehydrogenase short-chain) antibody is a crucial tool in studying the enzyme short-chain acyl-CoA dehydrogenase, which plays a pivotal role in mitochondrial fatty acid β-oxidation. This enzyme specifically catalyzes the dehydrogenation of short-chain acyl-CoA esters (e.g., butyryl-CoA), a critical step in energy production from fatty acids. Mutations in the ACADS gene are linked to short-chain acyl-CoA dehydrogenase deficiency (SCADD), an autosomal recessive disorder characterized by impaired fatty acid metabolism, leading to symptoms like hypoglycemia, muscle weakness, and developmental delays.

ACADS antibodies are primarily used in research and diagnostics to detect and quantify enzyme expression levels. They enable techniques such as Western blotting, immunohistochemistry, and ELISA to assess protein presence, tissue localization, and potential deficiencies. These antibodies aid in confirming SCADD diagnoses, understanding disease mechanisms, and evaluating therapeutic interventions in preclinical models. Commercially available as monoclonal or polyclonal variants, ACADS antibodies are validated for specificity to avoid cross-reactivity with medium- or long-chain acyl-CoA dehydrogenases. Recent studies also utilize these antibodies to explore ACADS roles beyond metabolic disorders, including its potential involvement in cancer and neurodegenerative diseases. Their application remains vital for advancing precision medicine approaches in inborn errors of metabolism.

客户数据及评论

折叠内容

大包装询价

×