Render Target: STATIC
Render Timestamp: 2024-12-13T11:25:24.531Z
Commit: 611277b6de3cd1bb065350b6ef8d63df412b7185
XML generation date: 2024-09-30 01:59:36.725
Product last modified at: 2024-12-05T14:45:18.846Z
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PDP - Template Name: Monoclonal Antibody
PDP - Template ID: *******c5e4b77
R Recombinant
Recombinant: Superior lot-to-lot consistency, continuous supply, and animal-free manufacturing.

COL6A1 (E9Q3P) Rabbit mAb #58232

Filter:
  • WB
  • IF

    Supporting Data

    REACTIVITY H M R
    SENSITIVITY Endogenous
    MW (kDa) 130
    Source/Isotype Rabbit IgG
    Application Key:
    • WB-Western Blotting 
    • IF-Immunofluorescence 
    Species Cross-Reactivity Key:
    • H-Human 
    • M-Mouse 
    • R-Rat 

    Product Information

    Product Usage Information

    Application Dilution
    Western Blotting 1:1000
    Simple Western™ 1:50 - 1:250
    Immunofluorescence (Frozen) 1:3200 - 1:6400
    Immunofluorescence (Immunocytochemistry) 1:3200 - 1:12800

    Storage

    Supplied in 10 mM sodium HEPES (pH 7.5), 150 mM NaCl, 100 µg/mL BSA, 50% glycerol, and less than 0.02% sodium azide. Store at –20°C. Do not aliquot the antibody.

    Protocol

    Specificity / Sensitivity

    COL6A1 (E9Q3P) Rabbit mAb recognizes endogenous levels of total COL6A1 protein. Non-specific cytoplasmic labeling of Purkinje cells in the cerebellum, acinar cells in pancreas, and tubules in testis may be observed by immunofluorescence.

    Species Reactivity:

    Human, Mouse, Rat

    Source / Purification

    Monoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Ile536 of human COL6A1 protein.

    Background

    Collagen type VI (ColVI) is a microfibrillar collagen found in the extracellular matrix (ECM) of muscles, bone, and connective tissues (1). ColVI consists of three main alpha chains: alpha 1, alpha 2, and alpha 3, which are encoded by the COL6A1, COL6A2, and COL6A3 genes, respectively. The three alpha chains form triple helix monomers and tetramers, which further assemble into a beaded microfilament network structure in the ECM (1,2). ColVI interacts with other ECM components, such as collagens, fibronectins, and perlecan, to support ECM mechanical sensing, anchoring the basement membrane to the surrounding ECM and inhibiting apoptosis and oxidative damage (3,4). Mutations in each of the ColVI genes (COL6A1, COL6A2, and COL6A3) result in defective ColVI assembly, causing Ullrich congenital muscular dystrophy (CMD) and Bethlem myopathy due to malformation of ECM structure (5,6). Knockout of COL6A1 in mice displays a mild myopathy and neurodegeneration associated with mitochondrial dysfunction, defective autophagy, and spontaneous apoptosis of muscle fibers (7,8). Increased COL6A1 in the ECM promotes tumor growth, metastasis, and therapeutic drug resistance (9-11).
    For Research Use Only. Not For Use In Diagnostic Procedures.
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