| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | IHC:1/100-1/200;IHF:1/50-1/200 | Human,Mouse,Rat |
| ICC | 1/50-1/200 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 咨询技术 | Human,Mouse,Rat |
| Aliases | Alpha 1 (VI) chain (61 AA); COL6A1; COL6A2; COL6A3; Collagen alpha 2(VI) chain; Collagen alpha 3(VI) chain; OPLL; PP3610;;Collagen VI |
| WB Predicted band size | Calculated MW: 109,344 kDa ; Observed MW: 147 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 | A synthesized peptide derived from human Collagen VI |
| Formulation | Purified antibody in PBS with 0.05% sodium azide,0.05% BSA and 50% glycerol. |
+ +
以下是关于 **Collagen VI 抗体** 的3篇参考文献的简要列举：
---
1. **文献名称**：*Collagen VI deficiency induces early onset myopathy in the mouse*
**作者**：Bonaldo P, et al.
**摘要**：该研究通过基因敲除小鼠模型，发现 **Collagen VI 缺失导致肌肉退化和线粒体功能障碍**，揭示了其在维持肌肉稳态中的作用。研究使用特异性抗体检测Collagen VI在细胞外基质的表达缺失与疾病表型的关联。
---
2. **文献名称**：*Collagen VI-related myopathies: Genetic and clinical heterogeneity*
**作者**：Ishikawa H, et al.
**摘要**：文章综述了 **Collagen VI基因突变(如COL6A1-3)引起的肌病(如Bethlem肌病和Ullrich先天性肌营养不良)**，强调了抗体在肌肉活检中诊断Collagen VI表达异常的重要性，并讨论了基因型与临床表型的关系。
---
3. **文献名称**：*Antibody-mediated inhibition of Collagen VI assembly ameliorates fibrosis in preclinical models*
**作者**：Zou Y, et al.
**摘要**：该研究开发了一种 **靶向Collagen VI的单克隆抗体**，在肺和皮肤纤维化小鼠模型中验证了其通过阻断Collagen VI沉积减轻纤维化的效果，为抗纤维化治疗提供了新策略。
---
4. **文献名称**：*Autophagy is defective in collagen VI muscular dystrophies through defective signaling*
**作者**：Grumati P, et al.
**摘要**：研究通过免疫荧光和Western blot分析发现，**Collagen VI缺陷导致自噬通路受损**，加剧肌肉萎缩。研究利用Collagen VI特异性抗体证实其表达水平与自噬标记物的相关性。
---
以上文献涵盖了Collagen VI抗体的应用场景(如疾病诊断、机制研究及治疗开发)及其在肌肉疾病和纤维化中的关键作用。
Collagen VI is a crucial extracellular matrix protein composed of three alpha chains (α1. α2. α3) encoded by the *COL6A1*, *COL6A2*, and *COL6A3* genes. It assembles into a unique microfibrillar network, providing structural support and regulating cellular processes like adhesion, migration, and signaling. Primarily expressed in muscles, skin, and cartilage, Collagen VI is vital for maintaining tissue integrity and homeostasis.
Antibodies targeting Collagen VI are essential tools in studying its expression, distribution, and role in diseases. Mutations in Collagen VI genes cause muscular disorders such as Bethlem myopathy and Ullrich congenital muscular dystrophy, characterized by muscle weakness and joint contractures. These antibodies enable detection of abnormal Collagen VI deposition in patient tissues, aiding diagnosis and mechanistic studies. Additionally, Collagen VI dysregulation is linked to fibrosis, cancer progression, and impaired wound healing. Researchers use Collagen VI antibodies in techniques like immunohistochemistry, Western blotting, and ELISA to explore its involvement in extracellular matrix remodeling, tumor microenvironment interactions, and therapeutic targeting.
Monoclonal and polyclonal Collagen VI antibodies are commercially available, often validated for specificity across species. Their applications extend to preclinical models, where they help evaluate Collagen VI’s contribution to disease pathology or tissue regeneration. As interest grows in matrix biology, Collagen VI antibodies remain pivotal in advancing both basic research and clinical diagnostics.
×
