CST, 52395S, COL6A1 (E9U3B) Rabbit Monoclonal Antibody

Monoclonal Antibody for studying COL6A1. Validated for Western Blotting,Immunohistochemistry (Paraffin),Immunofluorescence (Frozen). Available in 2 sizes. Highly specific and rigorously validated in-house, COL6A1 (E9U3B) Rabbit Monoclonal Antibody (CST #52395) is ready to ship. Product Usage Information Western Blotting: 1:1000 Immunohistochemistry (Paraffin): 1:50 - 1:200 Immunofluorescence (Frozen): 1:400 - 1:1600 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. For a carrier free (BSA and azide free) version of this product see product # 66606 . Protocol Available protocols: Western Blotting, Immunohistochemistry (Paraffin), Immunofluorescence (Frozen) Specificity / Sensitivity COL6A1 (E9U3B) Rabbit Monoclonal Antibody recognizes endogenous levels of total COL6A1 protein. This antibody is not recommended for immunohistochemical analysis of mouse tissues. Reactivity for immunofluorescence is mouse only. Species Reactivity: Human, Mouse, Rat Source / Purification Monoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues surrounding His1018 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 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 ( ) result in defective ColVI assembly, causing Ullrich congenital muscular dystrophy (CMD) and Bethlem myopathy due to malformation of ECM structure (5,6). Knockout of 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). Alternate Names alpha 1 (VI) chain (61 AA); BTHLM1; CO6A1; COL6A1; Collagen alpha-1(VI) chain; collagen type VI alpha 1 chain; collagen VI, alpha-1 polypeptide; collagen, type VI, alpha 1; epididymis secretory sperm binding protein; OPLL; UCHMD1 Specification REACTIVITY: H M R SENSITIVITY: Endogenous MW (kDa): 130 Source/Isotype: Rabbit IgG