概述

  • 产品名称Anti-Collagen VI抗体
    参阅全部 Collagen VI 一抗
  • 描述
    兔多克隆抗体to Collagen VI
  • 经测试应用适用于: WBmore details
  • 种属反应性
    与反应: Mouse, Human
    预测可用于: Rat, Horse, Cow, Dog, Pig, Chimpanzee, Macaque Monkey, Gorilla
  • 免疫原

    Synthetic peptide conjugated to KLH derived from within residues 150 - 250 of Human Collagen VI.

  • 阳性对照
    • This antibody gave a positive signal in the following tissue lysates: Human Skeletal Muscle; Human Skin; Human Heart as well as the following whole cell lysates: MEF1; NIH3T3; STO; WI38.

性能

  • 形式Liquid
  • 存放说明Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
  • 存储溶液pH: 7.40
    Preservative: 0.02% Sodium azide
    Constituent: PBS
    Note: Batches of this product that have a concentration < 1mg/ml may have BSA added as a stabilising agent. If you would like information about the formulation of a specific lot, please contact our scientific support team who will be happy to help.
  • 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 ab99249 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
WB Use a concentration of 1 µg/ml. Detects a band of approximately 147 kDa (predicted molecular weight: 108 kDa).

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
    • COL6A2 antibody
    • COL6A3 antibody
    • Collagen alpha 2(VI) chain antibody
    • Collagen alpha 3(VI) chain antibody
    • Collagen alpha-1(VI) chain antibody
    • Collagen type VI alpha 1 antibody
    • Collagen type VI alpha 2 antibody
    • Collagen type VI alpha 3 antibody
    • Collagen VI alpha 1 polypeptide antibody
    • Collagen VI alpha 2 polypeptide antibody
    • Collagen VI alpha 3 polypeptide antibody
    • CollagenVI antibody
    • Human mRNA for collagen VI alpha 2 C terminal globular domain antibody
    • OPLL antibody
    • PP3610 antibody
    see all

Anti-Collagen VI antibody 图像

  • All lanes : Anti-Collagen VI antibody (ab99249) at 1 µg/ml

    Lane 1 : Human skeletal muscle tissue lysate - total protein (ab29330)
    Lane 2 : Human thymus tissue lysate - total protein (ab30146)
    Lane 3 : Human heart tissue lysate - total protein (ab29431)
    Lane 4 : MEF1 (Mouse embryonic fibroblast cell line) Whole Cell Lysate
    Lane 5 : NIH 3T3 (Mouse embryonic fibroblast cell line) Whole Cell Lysate
    Lane 6 : STO (Mouse embryonic fibroblast cell line) Whole Cell Lysate
    Lane 7 : WI-38 whole cell lysate (ab3960)

    Lysates/proteins at 10 µg per lane.

    Secondary
    Goat Anti-Rabbit IgG H&L (HRP) preadsorbed (ab97080) at 1/5000 dilution
    Developed using the ECL technique

    Performed under reducing conditions.

    Predicted band size : 108 kDa
    Observed band size : 147 kDa (why is the actual band size different from the predicted?)
    Additional bands at : 53 kDa. We are unsure as to the identity of these extra bands.

    Exposure time : 2 minutes

Anti-Collagen VI antibody (ab99249)参考文献

This product has been referenced in:
  • Conejero-Goldberg C  et al. APOE2 enhances neuroprotection against Alzheimer's disease through multiple molecular mechanisms. Mol Psychiatry N/A:N/A (2014). Read more (PubMed: 24492349) »

See 1 Publication for this product

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