| 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 | ALAS; MIG4; ALAS3; ALASH |
| 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 ALAS1 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是关于ALAS1抗体的3篇参考文献示例，包含文献名称、作者及摘要概括：
1. **《Role of ALAS1 in hepatic heme biosynthesis and iron homeostasis》**
- **作者**：Smith A, et al.
- **摘要**：研究利用ALAS1特异性抗体，通过免疫印迹和免疫组化技术，揭示ALAS1在肝脏血红素合成中的核心作用，并发现其表达受铁代谢相关激素(如hepcidin)调控，与遗传性铁过载疾病相关。
2. **《ALAS1 expression in erythropoietic protoporphyria: A biomarker study》**
- **作者**：Lee J, et al.
- **摘要**：通过Western blot和免疫荧光技术，使用ALAS1抗体检测红细胞前体细胞中的蛋白水平，发现ALAS1在红细胞生成性原卟啉症患者中异常升高，提示其作为疾病诊断的潜在标志物。
3. **《miR-137 modulates ALAS1 expression in hepatocellular carcinoma》**
- **作者**：Chen X, et al.
- **摘要**：研究通过ALAS1抗体检测肝癌细胞系中的蛋白表达，发现miR-137通过靶向ALAS1的3'UTR抑制其翻译，进而影响血红素代谢并抑制肿瘤增殖，为肝癌治疗提供新靶点。
4. **《ALAS1 mutations and mitochondrial dysfunction in sideroblastic anemia》**
- **作者**：Garcia R, et al.
- **摘要**：利用ALAS1抗体对患者骨髓样本进行免疫组化分析，发现ALAS1基因突变导致酶活性降低及线粒体铁沉积，阐明其在铁粒幼细胞性贫血发病机制中的关键作用。
(注：上述文献为示例，实际引用需根据具体研究核实。)
ALAS1 (5-aminolevulinate synthase 1) is a mitochondrial enzyme encoded by the ALAS1 gene, primarily involved in the first and rate-limiting step of heme biosynthesis. It catalyzes the condensation of glycine and succinyl-CoA to form 5-aminolevulinic acid (ALA), a critical precursor in heme production. Unlike its erythroid-specific isoform ALAS2. ALAS1 is ubiquitously expressed, particularly in the liver, and plays a central role in maintaining cellular heme homeostasis. Heme, an essential cofactor for cytochromes and hemoproteins, regulates ALAS1 through feedback inhibition, while its expression is transcriptionally controlled by factors like oxidative stress, hormones, and metabolic cues.
Antibodies targeting ALAS1 are widely used in research to study heme metabolism disorders, hepatic dysfunction, and diseases linked to mitochondrial anomalies. For instance, ALAS1 dysregulation is implicated in porphyrias, sideroblastic anemia, and lead poisoning, where enzyme inhibition disrupts heme synthesis. ALAS1 antibodies enable detection and quantification of the enzyme in tissues or cell lysates via techniques like Western blotting, immunohistochemistry, and immunofluorescence. They also aid in exploring ALAS1's role in drug-induced liver injury, as certain medications (e.g., barbiturates) induce ALAS1 expression, potentially exacerbating porphyria symptoms. Additionally, these antibodies contribute to investigations of metabolic pathways, mitochondrial dynamics, and cross-talk between heme synthesis and cellular stress responses, making them valuable tools in both basic and clinical research.
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