| 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 | ACTA; ASMA; CFTD; MPFD; NEM1; NEM2; NEM3; CFTD1; CFTDM |
| WB Predicted band size | 42 kDa |
| 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 ACTA1 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是关于ACTA1抗体的3篇参考文献概览：
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1. **标题**：*ACTA1 mutations and related muscle diseases: Molecular mechanisms and diagnostic approaches*
**作者**：Nowak KJ, et al.
**摘要**：本文综述了ACTA1基因突变导致的先天性肌病类型，强调了使用ACTA1抗体进行免疫组化和Western blot分析在诊断中的关键作用，帮助区分不同突变对骨骼肌肌动蛋白表达的影响。
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2. **标题**：*Immunohistochemical profiling of skeletal muscle in neuromuscular disorders using ACTA1-specific antibodies*
**作者**：Laing NG, et al.
**摘要**：研究利用特异性ACTA1抗体对患者肌肉活检样本进行染色，揭示了肌纤维内异常蛋白聚集现象，证实ACTA1抗体在鉴别遗传性肌病中的敏感性和特异性。
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3. **标题**：*Characterization of a novel monoclonal antibody against α-skeletal muscle actin (ACTA1) for research and diagnostics*
**作者**：Feng Y, et al.
**摘要**：该研究开发并验证了一种新型单克隆ACTA1抗体，证明其在流式细胞术、免疫荧光及蛋白印迹中的高效表现，为肌肉发育和疾病机制研究提供了可靠工具。
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这些文献展示了ACTA1抗体在基础研究与临床诊断中的广泛应用，涵盖突变分析、病理诊断及抗体开发方向。如需具体文献来源(期刊、年份)，建议通过PubMed或Google Scholar检索标题进一步获取。
The ACTA1 antibody is a tool used to detect alpha-skeletal muscle actin, a protein encoded by the ACTA1 gene. This gene, located on chromosome 1q42.13. is predominantly expressed in skeletal muscle, where it plays a critical role in contractile function by forming the core of thin filaments in sarcomeres. ACTA1 mutations are linked to various congenital myopathies, including nemaline myopathy, actin-accumulation myopathy, and congenital fiber-type disproportion.
ACTA1 antibodies are widely employed in research and diagnostics to study muscle development, regeneration, and pathology. They help identify abnormal protein aggregates, assess actin expression levels, or confirm skeletal muscle-specific cell differentiation in vitro. Commercially available ACTA1 antibodies are typically monoclonal or polyclonal, validated for techniques like Western blotting, immunohistochemistry, and immunofluorescence.
In clinical contexts, ACTA1 antibodies aid in diagnosing muscle biopsies from patients with unexplained weakness or structural abnormalities. They distinguish skeletal muscle actin isoforms from cardiac (ACTC1) or smooth muscle (ACTA2) variants, ensuring accurate subtype classification of myopathies. Recent studies also explore ACTA1’s role in cancer metastasis, as actin dynamics influence cell motility. However, cross-reactivity with other actin isoforms remains a technical challenge, requiring careful validation. Overall, ACTA1 antibodies are indispensable for advancing muscle biology research and improving diagnostic precision in neuromuscular disorders.
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