| 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 | MMDS3; SPG74; C1orf69 |
| 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 | Synthetic peptide of human IBA57 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是3篇与IBA57抗体相关的文献示例(内容为虚构模拟，仅作格式参考)：
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1. **文献名称**: *"Role of IBA57 in Mitochondrial Iron-Sulfur Protein Biogenesis"*
**作者**: Smith, A. et al.
**摘要**: 研究阐明了IBA57蛋白在线粒体铁硫簇(Fe-S)组装中的关键作用。通过特异性抗体检测，发现IBA57与ISC系统相互作用，其缺失导致多种Fe-S酶活性下降，提示其在代谢疾病中的潜在机制。
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2. **文献名称**: *"Dysregulation of IBA57 Expression in Neurological Disorders"*
**作者**: Johnson, M. et al.
**摘要**: 利用抗IBA57抗体进行脑组织样本分析，发现IBA57在神经退行性疾病患者中表达显著降低，提示其可能通过影响线粒体功能参与疾病进程，为治疗靶点提供依据。
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3. **文献名称**: *"Functional Analysis of IBA57 Mutations Using Antibody-Based Assays"*
**作者**: Lee, H. et al.
**摘要**: 通过Western blot和免疫荧光技术，研究遗传性IBA57突变对蛋白稳定性和亚细胞定位的影响，证实突变导致线粒体Fe-S组装缺陷，与早发性脑病相关。
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如需真实文献，建议在PubMed或Google Scholar中搜索关键词“IBA57 antibody”或“IBA57 protein function”。
The IBA57 antibody is a crucial tool for studying the mitochondrial iron-sulfur cluster (Fe-S) assembly system. IBA57 is a mitochondrial protein involved in the biosynthesis of Fe-S clusters, essential cofactors for enzymes in critical cellular processes like electron transport, DNA repair, and metabolic regulation. It functions as part of the mitochondrial ISC machinery, working alongside other proteins (e.g., ISCU, NFU1) to assemble and transfer Fe-S clusters to target apoproteins. Mutations in the IBA57 gene are linked to mitochondrial disorders, such as multiple mitochondrial dysfunctions syndrome type 3 (MMDS3), characterized by severe neurological impairment and lactic acidosis.
Antibodies against IBA57 enable researchers to investigate its expression, localization, and interactions via techniques like Western blotting, immunofluorescence, and immunoprecipitation. These tools are vital for elucidating its role in Fe-S biogenesis and disease mechanisms. Studies using IBA57 antibodies have revealed reduced protein levels in patient-derived cells with pathogenic variants, confirming its clinical relevance. Additionally, they help explore IBA57's potential involvement in cancer, where altered Fe-S metabolism may drive tumor progression. Most IBA57 antibodies are raised in rabbits or mice using recombinant protein fragments, and their specificity is validated through knockdown/knockout controls. Overall, IBA57 antibodies are indispensable for advancing understanding of mitochondrial biology and associated pathologies.
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