Anti-Collagen III Antibody (RMAB-0250475)
Cat. No.: RMAB-0250475
Category: Primary Antibodies
INQUIRY
100 μL
Customer Size
Rabbit monoclonal to Collagen III
Product Features
| Isotype | IgG |
|---|---|
| Clonality | Monoclonal |
| Host Species | Rabbit |
| Clone Number | EPR17673 |
| Form | Liquid |
| Purity | Protein A purified |
| Species Reactivity | Human, Mouse, Rat |
| Immunogen | Recombinant fragment. |
| Applications | Flow Cyt (Intra), ICC/IF, IP, WB |
| Key Features | Produced recombinantly (animal-free) for high batch-to-batch consistency and long term security of supply; Rabbit monoclonal to Collagen III; Suitable for: Flow Cyt (Intra), ICC/IF, IP, WB; Reacts with: Mouse, Rat, Human |
Target Information
| Target Symbol | COL3A1 |
|---|---|
| Target Name | Collagen alpha-1(III) chain |
| UniProt ID | P02461 |
| Cellular Localization | Secreted > extracellular space > extracellular matrix. |
| Function | Collagen type III occurs in most soft connective tissues along with type I collagen. |
| Involvement in Disease | Defects in COL3A1 are a cause of Ehlers-Danlos syndrome type 3 (EDS3); also known as benign hypermobility syndrome. EDS is a connective tissue disorder characterized by hyperextensible skin, atrophic cutaneous scars due to tissue fragility and joint hyperlaxity. EDS3 is a form of Ehlers-Danlos syndrome characterized by marked joint hyperextensibility without skeletal deformity.Defects in COL3A1 are the cause of Ehlers-Danlos syndrome type 4 (EDS4). EDS is a connective tissue disorder characterized by hyperextensible skin, atrophic cutaneous scars due to tissue fragility and joint hyperlaxity. EDS4 is the most severe form of the disease. It is characterized by the joint and dermal manifestations as in other forms of the syndrome, characteristic facial features (acrogeria) in most patients, and by proneness to spontaneous rupture of bowel and large arteries. The vascular complications may affect all anatomical areas.Defects in COL3A1 are a cause of susceptibility to aortic aneurysm abdominal (AAA). AAA is a common multifactorial disorder characterized by permanent dilation of the abdominal aorta, usually due to degenerative changes in the aortic wall. Histologically, AAA is characterized by signs of chronic inflammation, destructive remodeling of the extracellular matrix, and depletion of vascular smooth muscle cells. |
| Post-translational Modifications | Proline residues at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains. O-linked glycan consists of a Glc-Gal disaccharide bound to the oxygen atom of a post-translationally added hydroxyl group. |
| Sequence Similarities | Belongs to the fibrillar collagen family. Contains 1 fibrillar collagen NC1 domain. Contains 1 VWFC domain. |
Storage & Shipping
| Storage Buffer | pH: 7.2; Preservative: 0.01% Sodium azide; Constituents: 0.05% BSA, 40% Glycerol, PBS |
|---|---|
| Storage & Shipping | Shipped at 4°C. Store at 4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle. |
For research use only. Not for clinical use.
