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Cell Signaling Col3a1 (E8d7r) Rabbit mAb (BSA And Azide Free)
List Price $865.00 Your Price $865.00
Cell Signaling Col3a1 (E8d7r) Rabbit mAb (BSA And Azide Free) - CSSPQ (Additional S&H or Hazmat Fees May Apply)
NETA PART: CSSPQ-98908SF
MFG.PART: 98908SF
UNSPSC: 12352203
Manufacturer: Cell Signaling
| Size | 100 µg |
| Reactivity | H |
| Sensitivity | Endogenous |
| Molecular Weight (kDa) | 200 |
| Source/Isotype | Rabbit IgG |
| Application/Dilution | {} |
| Storage | Supplied in 1X PBS, BSA and Azide Free. For standard formulation of this product see product #63034. |
| Specificity/Sensitivity | COL3A1 (E8D7R) XP® Rabbit mAb 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 comprised 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).COL3A1 (Collagen 3 alpha 1) is a major fibrillar collagen comprised 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 COL3A1 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). |
| SKU | CSSPQ-98908SF |
|---|---|
| Supplier Part Number | 98908SF |
| UM | EA |
| UNSPSC | 12352203 |
| Manufacturer | Cell Signaling |
| MSDS URL | Click here |
| Temperature | -20C |
| CountryOfOrigin | United States |
| ProductLine | CSSPQ |
| Qty | 1 |
| MinOrderQty | 1 |
| Weight | 7.000000 |
| Lead Time | 5 |
| Hazardous | N |
| Energy Star | No |
| Green | No |
| Controlled | N |