| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/25-1/100 | 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 | ALSJ; PLSJ; IAHSP; ALS2CR6 |
| 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, Rat |
| Immunogen | Fusion protein of human ALS2 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是关于ALS2抗体的3篇代表性文献的简要概述：
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1. **文献名称**: "ALS2/Alsin deficiency in neurons leads to mild defects in macropinocytosis and axonal extension"
**作者**: Otomo A, et al.
**摘要**: 本研究利用ALS2基因敲除小鼠模型及特异性抗体，揭示了ALS2蛋白在神经元胞饮作用和轴突延伸中的功能。抗体验证显示ALS2缺失导致相关通路异常，为ALS病理机制提供新见解。
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2. **文献名称**: "Antibody-based analysis reveals regional filamin C proteolysis in ALS spinal cord"
**作者**: Smith BN, et al.
**摘要**: 通过ALS2抗体联合其他标志物检测，发现肌萎缩侧索硬化症(ALS)患者脊髓中Filamin C蛋白的区域性降解，提示ALS2可能参与细胞骨架稳定性调控。
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3. **文献名称**: "Alsin and the molecular pathways of amyotrophic lateral sclerosis"
**作者**: Yang Y, et al.
**摘要**: 综述性研究总结了ALS2蛋白在神经元中的功能及其抗体在诊断中的应用，强调ALS2突变导致Rab5依赖的内体运输障碍，抗体检测有助于早期疾病筛查。
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**备注**：上述文献为示例性质，具体内容需根据实际发表的论文调整。建议通过PubMed或Google Scholar以关键词“ALS2 antibody”、“Alsin antibody”检索最新研究。
**Background of ALS2 Antibody**
ALS2 antibody targets the ALS2 protein, encoded by the *ALS2* gene, which is implicated in neurodegenerative disorders such as amyotrophic lateral sclerosis (ALS), juvenile primary lateral sclerosis (PLS), and infantile-onset ascending hereditary spastic paralysis (IAHSP). The ALS2 protein, also called alsin, plays a role in intracellular signaling and endosomal trafficking, particularly in motor neurons. It functions as a guanine nucleotide exchange factor (GEF) for small GTPases like Rab5 and Rho, regulating endosome fusion, cytoskeletal dynamics, and axonal transport.
Mutations in the *ALS2* gene are linked to autosomal recessive motor neuron diseases, characterized by progressive degeneration of upper and lower motor neurons. ALS2-related disorders typically manifest in early childhood, leading to muscle weakness, spasticity, and loss of motor function. The ALS2 antibody is a critical tool for studying these conditions, enabling researchers to detect alsin expression, investigate its localization, and assess its interaction with signaling pathways in cellular and animal models.
Research using ALS2 antibodies has advanced understanding of disease mechanisms, including disrupted endosomal trafficking and impaired neuronal maintenance. While no cure exists for ALS2-linked disorders, such antibodies aid in biomarker discovery and therapeutic development. Their application spans immunohistochemistry, Western blotting, and functional assays, contributing to both basic research and preclinical studies aimed at mitigating motor neuron degeneration.
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