| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/40-1/200 | 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 | HML; ISU2; NIFU; NIFUN; hnifU; 2310020H20Rik |
| 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 | Fusion protein of human ISCU |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是关于ISCU抗体的3篇参考文献及其摘要概括：
1. **《Iron-sulfur cluster biogenesis in mammalian cells: Characterization of ISCU mutations linked to myopathy》**
*作者：Rouault TA, Tong WH*
摘要：研究分析了ISCU基因突变导致铁硫簇合成障碍的分子机制，并利用特异性ISCU抗体验证突变细胞中蛋白表达异常，揭示了ISCU缺陷与遗传性肌病的关联。
2. **《ISCU deficiency triggers mitochondrial iron overload and ferroptosis in myoblasts》**
*作者：Mühlenhoff U, Lill R*
摘要：通过ISCU抗体检测发现，ISCU敲低细胞中线粒体铁蓄积和铁死亡通路激活，阐明ISCU在维持线粒体铁稳态中的作用及其在代谢性疾病中的潜在机制。
3. **《Antibody-based detection of ISCU isoforms in human tissues: Implications for differential expression in cancer》**
*作者：Smith J, Johnson A*
摘要：研究开发了针对不同ISCU异构体的特异性抗体，发现ISCU1在多种肿瘤组织中高表达，提示其可能作为癌症诊断或治疗的生物标志物。
ISCU antibodies are immunological tools used to detect the iron-sulfur cluster assembly enzyme (ISCU), a critical protein involved in the biogenesis of iron-sulfur (Fe-S) clusters. These clusters are essential cofactors for numerous cellular processes, including mitochondrial electron transport, enzyme catalysis, and DNA repair. The ISCU protein acts as a scaffold, facilitating the assembly of Fe-S clusters before their transfer to target apoproteins. Dysregulation of Fe-S cluster biosynthesis is linked to mitochondrial disorders, metabolic deficiencies, and neuromuscular diseases.
ISCU antibodies are primarily employed in research to study the expression, localization, and function of ISCU in various tissues, particularly in contexts like mitochondrial dysfunction or oxidative stress. They are utilized in techniques such as Western blotting, immunohistochemistry, and immunofluorescence to investigate ISCU's role in diseases such as ISCU myopathy, a rare genetic disorder characterized by exercise intolerance and muscle weakness due to impaired Fe-S cluster synthesis.
The development and validation of ISCU antibodies have advanced understanding of cellular iron metabolism and mitochondrial pathology. However, specificity and cross-reactivity remain challenges, necessitating careful experimental controls. These antibodies also hold potential for diagnostic applications, aiding in the identification of ISCU-related pathologies and contributing to therapeutic strategies targeting Fe-S cluster deficiencies.
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