| 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 | GE; NE; HLE; HNE; ELA2; SCN1; PMN-E |
| 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 ELANE |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
+ +
以下是关于ELANE抗体的3篇代表性文献及其摘要：
---
1. **文献名称**: *Mutations in the gene encoding neutrophil elastase in congenital and cyclic neutropenia*
**作者**: Horwitz MS, et al.
**摘要**: 该研究首次发现ELANE基因突变是先天性中性粒细胞减少症和周期性中性粒细胞减少症的关键病因，通过分析患者基因突变揭示了突变导致中性粒细胞成熟障碍的分子机制。
---
2. **文献名称**: *ELANE mutations in patients with severe congenital neutropenia: genotype-phenotype correlation*
**作者**: Germeshausen M, et al.
**摘要**: 研究通过统计ELANE突变类型(错义突变 vs. 无义突变)与临床表型的关联，发现特定突变类型(如跨膜结构域突变)与更严重的感染风险和白血病转化倾向相关。
---
3. **文献名称**: *Neutrophil elastase mutations in hereditary neutropenia: molecular mechanisms and clinical consequences*
**作者**: Nunoi H, et al.
**摘要**: 综述了ELANE突变导致中性粒细胞功能异常的病理机制，包括错误折叠蛋白在内质网的积累引发未折叠蛋白反应(UPR)，进而诱导细胞凋亡。
---
4. **文献名称**: *ELANE antibody-based detection of neutrophil elastase in clinical samples*
**作者**: Cowan TM, et al.
**摘要**: 开发了一种基于ELANE抗体的高灵敏度ELISA检测方法，用于快速诊断先天性中性粒细胞减少症，并验证其在患者外周血样本中的诊断价值。
---
这些文献涵盖了ELANE的遗传学机制、临床相关性及检测技术，可作为深入研究的基础。
ELANE antibody targets the protein encoded by the ELANE gene, also known as neutrophil elastase (NE). ELANE is a serine protease primarily stored in the azurophilic granules of neutrophils, playing a critical role in innate immunity by degrading pathogen-derived proteins and modulating inflammatory responses. It is involved in extracellular matrix remodeling, cytokine processing, and neutrophil extracellular trap (NET) formation. Dysregulation of ELANE activity is linked to several diseases. Mutations in the ELANE gene are a leading cause of congenital neutropenia (e.g., cyclic neutropenia and severe congenital neutropenia), where defective enzyme trafficking triggers endoplasmic reticulum stress, impairing neutrophil maturation. Additionally, excessive or uncontrolled ELANE release contributes to tissue damage in chronic inflammatory conditions like emphysema, cystic fibrosis, and chronic obstructive pulmonary disease (COPD).
ELANE antibodies are essential tools in research and diagnostics. They enable detection of ELANE expression in immune cells, assessment of protein localization, and evaluation of its role in disease mechanisms. These antibodies are widely used in techniques such as Western blotting, immunohistochemistry, and flow cytometry. In clinical settings, they aid in studying genetic mutations or monitoring neutrophil dysfunction. Therapeutic strategies targeting ELANE, including inhibitory antibodies or small-molecule inhibitors, are also under investigation to mitigate protease-mediated tissue injury in inflammatory diseases.
×
