Overview

  • Product name

    Anti-Collagen VI antibody [EPR17072] (HRP)
    See all Collagen VI primary antibodies
  • Description

    Rabbit monoclonal [EPR17072] to Collagen VI (HRP)
  • Host species

    Rabbit
  • Conjugation

    HRP
  • Tested applications

    Suitable for: WBmore details
  • Species reactivity

    Reacts with: Human
    Predicted to work with: Mouse, Rat
  • Immunogen

    Recombinant fragment within Human Collagen VI aa 1-250. The exact sequence is proprietary.
    Database link: P12109

  • Positive control

    • WB: Human skeletal muscle lysate and WI-38 whole cell lysates.
  • General notes

    Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMab® patents.

Properties

Applications

Our Abpromise guarantee covers the use of ab200596 in the following tested applications.

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

Application Abreviews Notes
WB 1/5000. Detects a band of approximately 147 kDa (predicted molecular weight: 109 kDa).

Target

  • Function

    Collagen VI acts as a cell-binding protein.
  • Involvement in disease

    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.
  • Sequence similarities

    Belongs to the type VI collagen family.
    Contains 3 VWFA domains.
  • Post-translational
    modifications

    Prolines at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains.
  • Cellular localization

    Secreted > extracellular space > extracellular matrix.
  • Information by UniProt
  • Database links

  • Alternative names

    • Alpha 1 (VI) chain (61 AA) antibody
    • CO6A1_HUMAN antibody
    • COL6A1 antibody
    • COL6A2 antibody
    • COL6A3 antibody
    • collagen 6 antibody
    • Collagen alpha 2(VI) chain antibody
    • Collagen alpha 3(VI) chain antibody
    • Collagen alpha-1(VI) chain antibody
    • collagen six 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

Images

  • All lanes : Anti-Collagen VI antibody [EPR17072] (HRP) (ab200596) at 1/5000 dilution

    Lane 1 : Skeletal Muscle (Human) Tissue Lysate - adult normal tissue at 20 µg
    Lane 2 : WI38 (Human lung fibroblast cell line) Whole Cell Lysate at 10 µg

    Developed using the ECL technique.

    Performed under reducing conditions.

    Predicted band size: 109 kDa
    Observed band size: 147 kDa
    why is the actual band size different from the predicted?


    Exposure time: 3 minutes


    This blot was produced using a 4-12% Bis-tris gel under the MOPS buffer system. The gel was run at 200V for 50 minutes before being transferred onto a Nitrocellulose membrane at 30V for 70 minutes. The membrane was then blocked for an hour using 3% milk before being incubated with ab200596 overnight at 4°C. Antibody binding was visualised using ECL development solution ab133406.

References

ab200596 has not yet been referenced specifically in any publications.

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