Anti-Collagen IV Antibody (RMAB-0250478)
Cat. No.: RMAB-0250478
Category: Primary Antibodies
INQUIRY
100 μL
Customer Size
Rabbit monoclonal to Collagen IV
Product Features
| Isotype | IgG |
|---|---|
| Clonality | Monoclonal |
| Host Species | Rabbit |
| Clone Number | EPR20966 |
| Form | Liquid |
| Purity | Protein A purified |
| Species Reactivity | Human |
| Immunogen | Synthetic peptide. |
| Applications | IHC-P, WB |
| Key Features | Produced recombinantly (animal-free) for high batch-to-batch consistency and long term security of supply; Rabbit monoclonal to Collagen IV; Suitable for: IHC-P, WB; Knockout validated; Reacts with: Human |
Target Information
| Target Symbol | COL4A1 |
|---|---|
| Target Name | Collagen alpha-1(IV) chain |
| UniProt ID | P02462 |
| Cellular Localization | Secreted > extracellular space > extracellular matrix > basement membrane. |
| Function | Type IV collagen is the major structural component of glomerular basement membranes (GBM), forming a 'chicken-wire' meshwork together with laminins, proteoglycans and entactin/nidogen.Arresten, comprising the C-terminal NC1 domain, inhibits angiogenesis and tumor formation. The C-terminal half is found to possess the anti-angiogenic activity. Specifically inhibits endothelial cell proliferation, migration and tube formation. Inhibits expression of hypoxia-inducible factor 1alpha and ERK1/2 and p38 MAPK activation. Ligand for alpha1/beta1 integrin. |
| Involvement in Disease | Defects in COL4A1 are a cause of brain small vessel disease with hemorrhage (BSVDH). Brain small vessel diseases underlie 2 to 3 percent of ischemic strokes and a larger proportion of intracerebral hemorrhages. Inheritance is autosomal dominant.Defects in COL4A1 are the cause of hereditary angiopathy with nephropathy aneurysms and muscle cramps (HANAC). The clinical renal manifestations include hematuria and bilateral large cysts. Histologic analysis revealed complex basement membrane defects in kidney and skin. The systemic angiopathy appears to affect both small vessels and large arteries.Defects in COL4A1 are a cause of porencephaly familial (PCEPH). Porencephaly is a term used for any cavitation or cerebrospinal fluid-filled cyst in the brain. Porencephaly type 1 is usually unilateral and results from focal destructive lesions such as fetal vascular occlusion or birth trauma. Type 2, or schizencephalic porencephaly, is usually symmetric and represents a primary defect or arrest in the development of the cerebral ventricles. |
| Post-translational Modifications | Lysines at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in all cases and bind carbohydrates. Prolines at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains. Type IV collagens contain numerous cysteine residues which are involved in inter- and intramolecular disulfide bonding. 12 of these, located in the NC1 domain, are conserved in all known type IV collagens. The trimeric structure of the NC1 domains is stabilized by covalent bonds between Lys and Met residues. Proteolytic processing produces the C-terminal NC1 peptide, arresten. |
| Domain | Alpha chains of type IV collagen have a non-collagenous domain (NC1) at their C-terminus, frequent interruptions of the G-X-Y repeats in the long central triple-helical domain (which may cause flexibility in the triple helix), and a short N-terminal triple-helical 7S domain. |
| Sequence Similarities | Belongs to the type IV collagen family. Contains 1 collagen IV NC1 (C-terminal non-collagenous) 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.
