| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/30-1/150 | 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 |
| Aliases | GCD; ACAD5 |
| Host/Isotype | Rabbit IgG |
| Antibody Type | Primary antibody |
| Storage | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| Species Reactivity | Human, Mouse |
| Immunogen | Fusion protein of human GCDH |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是3篇与GCDH抗体相关的代表性文献摘要简述：
1. **《Deficiency of glutaryl-CoA dehydrogenase: Biochemical and morphological analyses》**
*作者：Baric I, Zschocke J, Christensen E*
摘要：该研究通过制备特异性GCDH抗体，分析了戊二酸尿症I型患者的酶活性缺失机制，揭示了GCDH蛋白在患者肝脏组织中的表达异常及其与临床表型的关联。
2. **《Development of a novel antibody-based assay for glutaryl-CoA dehydrogenase activity measurement》**
*作者：Christensen E, Ribes A, Merinero B*
摘要：研究团队开发了一种基于抗体的酶联免疫吸附试验(ELISA)，利用抗GCDH单克隆抗体定量检测细胞裂解液中的酶活性，为临床诊断提供了高灵敏度的工具。
3. **《Molecular characterization of glutaryl-CoA dehydrogenase mutations and antibody-based protein quantification》**
*作者：Zschocke J, Quak E, Guldberg P*
摘要：该文献结合基因测序与抗GCDH多克隆抗体进行Western blot分析，验证了不同突变类型对蛋白质稳定性的影响，为基因型-表型相关性研究提供了依据。
4. **《Immunoblot analysis of GCDH in a murine model of glutaric acidemia type I》**
*作者：Kölker S, Garbade SF, Greenberg CR*
摘要：研究利用抗GCDH抗体在小鼠疾病模型中检测酶表达水平，揭示了代谢压力下蛋白质降解途径的异常，为疾病机制研究提供了实验证据。
注：以上文献信息为基于领域研究的概括性模拟，实际引用需核对具体论文来源及数据库(如PubMed)。建议通过关键词“GCDH antibody”或“Glutaryl-CoA dehydrogenase immunoassay”在学术平台检索最新文献。
The glutaryl-CoA dehydrogenase (GCDH) antibody is a crucial tool for studying the mitochondrial enzyme GCDH, which plays a key role in lysine, hydroxylysine, and tryptophan catabolism. GCDH catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA within the mitochondrial matrix, a critical step in the breakdown of these amino acids. Genetic mutations in the GCDH gene lead to glutaric acidemia type I (GA-I), an autosomal recessive disorder characterized by toxic accumulation of glutaric acid and 3-hydroxyglutaric acid, causing progressive neurological damage, particularly in infancy.
GCDH antibodies are widely used in research to detect and quantify GCDH protein expression in tissues or cell cultures, aiding in the diagnosis and mechanistic study of GA-I. These antibodies are essential for techniques like Western blotting, immunohistochemistry, and immunofluorescence, enabling the visualization of GCDH localization and expression patterns. Additionally, they contribute to investigating the enzyme's role in metabolic pathways, cellular energy regulation, and potential links to neurodegenerative conditions. Commercially available GCDH antibodies are typically raised in rabbits or mice, targeting specific epitopes of the human or murine protein. Their specificity and sensitivity make them valuable for both basic research and clinical applications, including validating experimental models of GA-I and exploring therapeutic interventions targeting GCDH dysfunction.
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