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Rabbit Polyclonal COL6A2 Antibody

  • 中文名: COL6A2抗体
  • 别    名: Collagen alpha-2(VI) chain, COL6A2
货号: IPDX31632
Price: ¥1180
数量:
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验证与应用

应用及物种
WB 1/1000 Human,Mouse,Rat
IF 咨询技术 Human,Mouse,Rat
IHC 咨询技术 Human,Mouse,Rat
ICC 技术咨询 Human,Mouse,Rat
FCM 咨询技术 Human,Mouse,Rat
Elisa 咨询技术 Human,Mouse,Rat

产品详情

AliasesCollagen alpha-2(VI) chain, COL6A2
Entrez GeneID1292
WB Predicted band size108.6kDa
Host/IsotypeRabbit IgG
Antibody TypePrimary antibody
StorageStore at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.
Species ReactivityHuman
ImmunogenThis COL6A2 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 719-747 amino acids from the C-terminal region of human COL6A2.
FormulationPurified antibody in PBS with 0.05% sodium azide.

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参考文献

以下是3篇与COL6A2抗体相关的文献示例(内容为模拟概括,仅供参考):

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1. **文献名称**: *Autoantibodies against collagen VI in patients with connective tissue diseases*

**作者**: Briñas L, et al.

**摘要**: 研究检测了结缔组织病患者血清中COL6A2抗体的存在,发现其与特定亚型的肌营养不良和自身免疫性疾病相关,提示该抗体可能作为疾病分型的生物标志物。

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2. **文献名称**: *COL6A2 mutations and antibody-based detection in Ullrich congenital muscular dystrophy*

**作者**: Nadeau A, et al.

**摘要**: 通过免疫组织化学和Western blot分析,验证COL6A2抗体在诊断Ullrich先天性肌营养不良中的特异性,揭示了COL6A2基因突变导致胶原蛋白异常表达的分子机制。

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3. **文献名称**: *Extracellular matrix remodeling in fibrosis: Role of COL6A2 and therapeutic antibody targeting*

**作者**: Zou Y, et al.

**摘要**: 探讨COL6A2在纤维化疾病中的作用,利用特异性抗体阻断胶原VI功能,证明其在抑制病理性的细胞外基质沉积中的潜在治疗价值。

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4. **文献名称**: *Collagen VI-related myopathies: Diagnostic utility of antibody-based assays*

**作者**: Lampe AK, Bushby KM

**摘要**: 综述总结了COL6A2抗体在Bethlem肌病等胶原VI相关肌病中的临床应用,强调抗体检测对早期诊断和疾病监测的重要性。

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注:以上文献信息为示例性概括,实际文献需通过PubMed或学术数据库检索确认。

背景信息

The COL6A2 antibody targets the α2 chain of collagen VI, a key extracellular matrix protein encoded by the *COL6A2* gene. Collagen VI plays a critical role in maintaining tissue integrity by forming microfibrillar networks that link cells to the matrix, particularly in muscles, skin, and connective tissues. Mutations in *COL6A2* are associated with collagen VI-related disorders, such as Ullrich congenital muscular dystrophy (UCMD) and Bethlem myopathy, characterized by muscle weakness, joint contractures, and skin abnormalities. COL6A2 antibodies are essential tools for studying these diseases, enabling researchers to visualize collagen VI distribution in tissues, assess expression levels in patient samples, and investigate disease mechanisms in cellular or animal models. These antibodies are widely used in techniques like immunohistochemistry, Western blotting, and immunofluorescence. Recent studies also explore their potential in therapeutic strategies, such as monitoring collagen VI restoration in gene therapy or drug trials. Challenges include ensuring antibody specificity due to structural similarities among collagen VI chains and variability in post-translational modifications. Ongoing research aims to refine antibody validation protocols and expand applications in diagnostics and regenerative medicine.

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