概述

  • 产品名称Anti-COL6A1抗体
    参阅全部 COL6A1 一抗
  • 描述
    兔多克隆抗体to COL6A1
  • 经测试应用适用于: IHC-Pmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    Synthetic peptide derived from the internal region of Human COL6A1.

  • 阳性对照
    • Human heart tissue

性能

  • 形式Liquid
  • 存放说明Shipped at 4°C. Store at -20°C. Stable for 12 months at -20°C.
  • 存储溶液pH: 7.40
    Preservative: 0.02% Sodium azide
    Constituents: 49% PBS, 50% Glycerol, 0.88% Sodium chloride
  • Concentration information loading...
  • 纯度Immunogen affinity purified
  • 克隆多克隆
  • 同种型IgG
  • 研究领域

应用

Our Abpromise guarantee covers the use of ab111819 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

应用 Ab评论 说明
IHC-P 1/50 - 1/100. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.

靶标

  • 功能Collagen VI acts as a cell-binding protein.
  • 疾病相关Defects in COL6A1 are a cause of Bethlem myopathy (BM) [MIM:158810]. BM is a rare autosomal dominant proximal myopathy characterized by early childhood onset (complete penetrance by the age of 5) and joint contractures most frequently affecting the elbows and ankles.
    Defects in COL6A1 are a cause of Ullrich congenital muscular dystrophy (UCMD) [MIM:254090]; also known as Ullrich scleroatonic muscular dystrophy. UCMD is an autosomal recessive congenital myopathy characterized by muscle weakness and multiple joint contractures, generally noted at birth or early infancy. The clinical course is more severe than in Bethlem myopathy.
  • 序列相似性Belongs to the type VI collagen family.
    Contains 3 VWFA domains.
  • 翻译后修饰Prolines at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains.
  • 细胞定位Secreted > extracellular space > extracellular matrix.
  • Information by UniProt
  • 数据库链接
  • 别名
    • Alpha 1 (VI) chain (61 AA) antibody
    • CO6A1_HUMAN antibody
    • COL6A1 antibody
    • Collagen alpha-1(VI) chain antibody
    • Collagen VI, alpha 1 polypeptide antibody
    • Collagen, intimal short chain collagen antibody
    • Collagen, type VI, alpha 1 antibody
    • OPLL antibody
    • OTTHUMP00000115501 antibody
    see all

Anti-COL6A1 antibody 图像

  • ab111819 at 1/50 dilution staining COL6A1 in paraffin-embedded Human heart tissue by Immunohistochemistry. The image on the right is treated with synthesized peptide.

Anti-COL6A1 antibody (ab111819)参考文献

ab111819 has not yet been referenced specifically in any publications.

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