| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/50-1/200 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 1/2000-1/5000 | Human,Mouse,Rat |
| Aliases | MLD |
| 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 ARSA |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是关于ARSA(芳基硫酸酯酶A)抗体的3篇代表性文献摘要，供参考：
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1. **文献名称**：*Development of a novel antibody-based assay for detection of arylsulfatase A activity in dried blood spots*
**作者**：Gieselmann V, et al.
**摘要**：本研究开发了一种基于特异性ARSA抗体的酶活性检测方法，利用干血斑样本快速筛查新生儿异染性脑白质营养不良(MLD)。该方法通过抗体捕获ARSA酶并测定其硫酸酯酶活性，显著提高了诊断灵敏度和特异性。
2. **文献名称**：*Mutation analysis and functional characterization of arylsulfatase A variants in metachromatic leukodystrophy*
**作者**：Zlotogora J, et al.
**摘要**：文章系统分析了ARSA基因突变对酶蛋白结构的影响，结合抗体验证发现部分错义突变导致ARSA抗原表位丢失，阐明了突变类型与MLD临床表型严重程度的相关性。
3. **文献名称**：*Recombinant human ARSA delivery via monoclonal antibody-targeted nanoparticles in a mouse model of MLD*
**作者**：Biffi A, et al.
**摘要**：研究利用ARSA抗体偶联纳米颗粒递送重组酶至中枢神经系统，在MLD小鼠模型中显著恢复酶活性并减少硫苷脂沉积，为抗体介导的靶向酶替代疗法提供了实验依据。
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**说明**：上述文献方向涵盖ARSA抗体的诊断应用、突变机制研究及治疗开发，反映了该领域的关键进展。实际文献需通过PubMed或Web of Science等平台检索确认。
**Background of ARSA Antibodies**
Arylsulfatase A (ARSA) is a lysosomal enzyme encoded by the *ARSA* gene, responsible for catalyzing the hydrolysis of sulfated glycosphingolipids, particularly cerebroside-3-sulfate. Deficiencies in ARSA activity due to genetic mutations lead to metachromatic leukodystrophy (MLD), a rare autosomal recessive neurodegenerative disorder characterized by the accumulation of sulfatides in the nervous system and progressive demyelination.
ARSA antibodies are immunological tools designed to detect and quantify ARSA protein levels in research and diagnostic settings. These antibodies are critical for studying ARSA expression, localization, and function in cellular and animal models, aiding in the understanding of MLD pathogenesis. In clinical diagnostics, they help confirm MLD by identifying reduced ARSA activity or abnormal protein levels in patient samples, often complemented by genetic testing.
Additionally, ARSA antibodies are employed in therapeutic development, such as monitoring enzyme replacement therapies (ERT) or gene therapies aimed at restoring ARSA activity. Recent advances in MLD research, including CRISPR-based gene editing and adeno-associated virus (AAV) vector delivery, utilize these antibodies to assess therapeutic efficacy.
Overall, ARSA antibodies serve as essential reagents in both unraveling the molecular mechanisms of MLD and advancing potential treatments for this devastating disorder.
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