| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/15-1/50 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 1/1000-1/2000 | Human,Mouse,Rat |
| Aliases | ASL; AMPS; ASASE |
| 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 ADSL |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是关于ADSL抗体的模拟参考文献示例(注：文献为虚构示例，仅作格式参考)：
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1. **标题**: *Development of a Monoclonal Antibody for Human Adenylosuccinate Lyase (ADSL) Detection*
**作者**: Smith J, Lee K, et al.
**摘要**: 研究团队开发了一种高特异性的单克隆抗体，用于检测人类ADSL蛋白的表达。该抗体通过Western blot和免疫组化验证，成功应用于ADSL缺乏症患者的酶水平定量分析，为临床诊断提供了新工具。
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2. **标题**: *ADSL Deficiency Screening: Antibody-Based ELISA Method for Newborns*
**作者**: Garcia R, Müller T.
**摘要**: 提出一种基于ADSL抗体的ELISA检测方法，用于新生儿ADSL缺乏症的大规模筛查。研究显示该方法灵敏度达95%，可替代传统酶活性检测，降低诊断成本。
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3. **标题**: *Tissue-Specific Expression of ADSL in Mammalian Brain: Insights from Immunohistochemistry*
**作者**: Chen L, Wang H.
**摘要**: 利用ADSL多克隆抗体研究小鼠脑组织中ADSL的分布，发现其在小脑和海马体高表达，提示其在神经发育中的潜在作用，为代谢性脑病机制研究提供依据。
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4. **标题**: *ADSL Antibody Cross-Reactivity in Autoimmune Disorders: A Case Study*
**作者**: Patel S, et al.
**摘要**: 探讨ADSL抗体在自身免疫疾病患者中的交叉反应性，发现部分系统性红斑狼疮患者血清中存在针对ADSL的自身抗体，可能与其代谢异常相关。
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**备注**：以上文献为示例性内容，实际研究中请通过学术数据库(如PubMed、Web of Science)检索真实文献。建议结合关键词“ADSL antibody”和“adenylosuccinate lyase deficiency”进行深入查询。
Adenylosuccinate lyase (ADSL) is a critical enzyme in the purine biosynthesis pathway, catalyzing two distinct reactions: the conversion of succinylaminoimidazole carboxamide ribotide (SAICAR) to aminoimidazole carboxamide ribotide (AICAR) and the cleavage of adenylosuccinate (S-AMP) to adenosine monophosphate (AMP). Mutations in the ADSL gene are linked to rare autosomal recessive metabolic disorders, such as ADSL deficiency (OMIM #608222), characterized by severe neurological impairments, developmental delays, and autism-like symptoms. ADSL deficiency disrupts cellular energy metabolism and purine recycling, leading to the accumulation of toxic metabolites like succinylpurines in bodily fluids, which serve as diagnostic biomarkers.
ADSL antibodies are essential tools for studying this enzyme's expression, localization, and function in both normal and disease contexts. They enable researchers to detect ADSL protein levels via techniques like Western blotting, immunohistochemistry, or immunofluorescence, aiding in the identification of pathogenic mutations or altered enzyme activity. Additionally, these antibodies support investigations into the molecular mechanisms underlying ADSL-related disorders and potential therapeutic strategies. Commercially available ADSL antibodies are typically raised against specific epitopes of the human protein, validated for specificity in various model systems. Understanding ADSL's role in cellular metabolism and its association with neurodevelopmental disorders continues to drive interest in developing targeted diagnostics and treatments, positioning ADSL antibodies as vital reagents in both basic research and clinical applications.
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