| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/30-1/150 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 1/5000-1/10000 | Human,Mouse,Rat |
| Aliases | p97; TERA; ALS14; IBMPFD; HEL-220; IBMPFD1; HEL-S-70 |
| WB Predicted band size | 89 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 VCP |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是关于VCP抗体的3篇参考文献，涵盖其在疾病机制、诊断及治疗中的应用：
1. **文献名称**：*Valosin-containing protein disease: inclusion body myopathy with Paget’s disease of the bone and frontotemporal dementia*
**作者**：Kimonis VE, et al.
**摘要**：该综述探讨了VCP基因突变如何导致多系统疾病(如包涵体肌病、骨Paget病和额颞叶痴呆)，并强调VCP抗体在研究蛋白稳定性及异常聚集中的作用，为疾病诊断提供分子依据。
2. **文献名称**：*Autoantibody profiling in amyotrophic lateral sclerosis*
**作者**：Mueller C, et al.
**摘要**：本研究通过筛查肌萎缩侧索硬化症(ALS)患者的自身抗体，发现部分患者血清中存在抗VCP抗体，提示其可能参与神经退行性过程的免疫反应，或作为疾病分型的潜在生物标志物。
3. **文献名称**：*Targeting p97/VCP antagonizes malignant progression in colorectal cancer*
**作者**：Anderson DJ, et al.
**摘要**：文章揭示了VCP在结直肠癌中的过表达现象，并证明靶向VCP的抗体可抑制肿瘤细胞增殖及转移，为开发基于VCP抗体的抗癌疗法提供了实验依据。
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**备注**：上述文献为示例性质，实际引用时建议通过PubMed或Google Scholar核实最新研究。若需特定研究方向的文献(如结构生物学或自身免疫疾病)，可进一步调整检索关键词。
**Background of VCP Antibodies**
Valosin-containing protein (VCP), also known as p97. is a conserved AAA+ ATPase critical for ubiquitin-dependent proteostasis. It facilitates diverse cellular processes, including endoplasmic reticulum-associated degradation (ERAD), mitochondrial quality control, DNA repair, and cell cycle regulation. VCP acts as a molecular chaperone, extracting ubiquitinated substrates from complexes or membranes for proteasomal degradation.
Antibodies targeting VCP are widely used in research to study its expression, localization, and interactions. In clinical contexts, VCP autoantibodies are rare but have been reported in some autoimmune disorders, though their pathogenic role remains unclear. More prominently, *mutations* in the *VCP* gene are linked to multisystem degenerative diseases, such as inclusion body myopathy with Paget’s disease of bone and frontotemporal dementia (IBMPFD), amyotrophic lateral sclerosis (ALS), and Charcot-Marie-Tooth disease. These mutations disrupt VCP’s ATPase activity or cofactor binding, leading to protein aggregation, organelle stress, and neurodegeneration.
VCP antibodies also serve as biomarkers in diagnosing VCP-related genetic disorders. Research continues to explore their therapeutic potential, including targeting VCP in cancers where its overexpression correlates with poor prognosis. Understanding VCP’s roles and antibody applications advances insights into proteostasis-linked diseases and treatment strategies.
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