| 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 |
| Aliases | Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial, SBCAD, 2-methyl branched chain acyl-CoA dehydrogenase, 2-MEBCAD, 2-methylbutyryl-coenzyme A dehydrogenase, 2-methylbutyryl-CoA dehydrogenase, ACADSB |
| Entrez GeneID | 36 |
| WB Predicted band size | 47.5kDa |
| 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 |
| Immunogen | This ACADSB antibody is generated from a rabbit immunized with a KLH conjugated synthetic peptide between 239-273 amino acids from the Central region of human ACADSB. |
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以下是3篇关于ACADSB抗体的参考文献及其摘要概括：
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1. **文献名称**: *2-Methylbutyryl-CoA Dehydrogenase Deficiency: A Novel Inborn Error of L-Isoleucine Metabolism*
**作者**: Sutton VR, et al.
**摘要**: 本研究首次报道了由ACADSB基因突变引起的2-甲基丁酰辅酶A脱氢酶缺乏症。通过患者成纤维细胞分析，利用ACADSB抗体进行蛋白质印迹(Western blot)，发现患者酶蛋白表达显著降低，揭示了该代谢缺陷的分子机制。
2. **文献名称**: *ACADSB Antibodies as a Diagnostic Tool for Short/Branched-Chain Acyl-CoA Dehydrogenase Deficiency*
**作者**: Pedersen CB, et al.
**摘要**: 研究验证了ACADSB抗体在临床诊断中的应用，通过免疫分析检测患者血液和成纤维细胞中的酶水平，证实其可作为快速筛查支链脂肪酸代谢异常的生物标志物，并关联了基因型与表型的关系。
3. **文献名称**: *Tissue-Specific Expression of Short/Branched-Chain Acyl-CoA Dehydrogenase in Mice*
**作者**: Okamura-Ikeda K, et al.
**摘要**: 利用ACADSB特异性抗体进行免疫组化分析，揭示了该酶在小鼠肝脏、肾脏和肌肉中的分布模式，并探讨其在支链氨基酸代谢中的组织特异性功能，为研究代谢疾病提供了病理学基础。
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这些文献涵盖了ACADSB抗体在疾病诊断、蛋白质表达分析及组织分布研究中的关键应用。如需具体全文，建议通过PubMed或SciHub等平台检索标题获取。
The ACADSB (Acyl-CoA Dehydrogenase Short/Branched Chain) antibody is a key tool for studying the ACADSB enzyme, which plays a critical role in mitochondrial fatty acid β-oxidation. ACADSB specifically catalyzes the dehydrogenation of short/branched-chain acyl-CoA substrates, such as 2-methylbutyryl-CoA and isobutyryl-CoA, during the breakdown of branched-chain amino acids (e.g., valine) and dietary fatty acids. Mutations in the ACADSB gene are linked to 2-methylbutyryl-CoA dehydrogenase deficiency (2-MBCD), a rare autosomal recessive metabolic disorder characterized by elevated levels of specific acylcarnitines and organic acids, with symptoms ranging from asymptomatic presentations to severe neonatal metabolic crises.
ACADSB antibodies are widely used in research to detect and quantify ACADSB protein expression in tissues or cells, aiding in the investigation of its physiological roles, disease mechanisms, and interactions within metabolic pathways. These antibodies are validated for applications like Western blotting, immunohistochemistry, and immunofluorescence. Specificity and sensitivity are critical, as cross-reactivity with other acyl-CoA dehydrogenases (e.g., ACADS or ACADM) could lead to misleading results. Researchers also employ ACADSB antibodies to explore its potential involvement in metabolic syndromes, cancer, or drug-induced mitochondrial dysfunction, underscoring its broader relevance in biomedical studies. Proper validation using knockout controls or siRNA-mediated silencing is essential to ensure antibody reliability.
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