CST, 63034T, COL3A1 (E8D7R) Rabbit Monoclonal Antibody

Monoclonal Antibody for studying COL3A1. Validated for Western Blotting,Immunohistochemistry (Paraffin),Immunofluorescence (Immunocytochemistry). Available in 2 sizes. Highly specific and rigorously validated in-house, COL3A1 (E8D7R) Rabbit Monoclonal Antibody (CST #63034) is ready to ship. Product Usage Information Western Blotting: 1:1000 Immunohistochemistry (Paraffin): 1:250 - 1:1000 Immunofluorescence (Immunocytochemistry): 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. For a carrier-free (BSA and azide free) version of this product see product # 98908 . Protocol Available protocols: Western Blotting, Immunohistochemistry (Paraffin), Immunofluorescence (Immunocytochemistry) Specificity / Sensitivity COL3A1 (E8D7R) Rabbit Monoclonal Antibody recognizes endogenous levels of total COL3A1 protein. Species Reactivity: Human Source / Purification Monoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues near the carboxy terminus of human COL3A1 protein. Background The extracellular matrix (ECM) is a complex network of macromolecules that provides structural tissue support to cells in the basement membrane and interstitial matrix. It is composed of many molecules including proteins, glycoproteins, proteoglycans, and polysaccharides (1,2). One of the major proteins that comprises the ECM, and the human body, is collagen. Collagens are a large family of proteins. They are trimeric molecules composed of three alpha polypeptide chains that form a triple helix structure that is characteristic of all collagens (3). The large family of collagens is divided into three subgroups: fibrillar collagens, non-fibril forming collagens, and fibril-associated collagens. These subgroups differ in their structure and supramolecular assembly (3). Collagen 3 alpha 1 (COL3A1) is a major fibrillar collagen composed of three identical alpha-1 chains. It is present in most soft tissues, along with COL1A1, and is particularly high in tissues exhibiting elastic properties, such as the cardiac arterial wall and skin (4). Heterozygous mutations in the gene that cause missense mutation of a critical glycine residue in the triple helical domain of the alpha-1 chain result in vascular Ehlers-Danlos syndrome (vEDS). This mutation interferes with the ability of the alpha-1 chain to form collagen fibrils and thus disrupts macromolecular assembly of collagen fibers. vEDS is a severe and life-threatening disease as patients have a propensity for rupture of large arteries (4). Increased amounts of type III COL3A1 are found in many fibrotic conditions, such as lung, liver, kidney fibrosis, and systemic sclerosis (5,6). Alternate Names alpha-1 type III collagen; alpha1 (III) collagen; CO3A1; COL3A1; Collagen alpha-1(III) chain; Collagen III; Collagen Type III; collagen type III alpha 1 chain; collagen, fetal; collagen, type III, alpha 1; EDS4A; EDSVASC; Ehlers-Danlos syndrome type IV, autosomal dominant; FLJ34534; PMGEDSV; Type III Collagen Specification REACTIVITY: H SENSITIVITY: Endogenous MW (kDa): 200 Source/Isotype: Rabbit IgG