Collagen Type III [PT0118] mouse mAb
Ucallm_WM1521-50
Price range: $268.00 through $328.00
Product details
Background:
collagen type III alpha 1 chain(COL3A1) Homo sapiens This gene encodes the pro-alpha1 chains of type III collagen, a fibrillar collagen that is found in extensible connective tissues such as skin, lung, uterus, intestine and the vascular system, frequently in association with type I collagen. Mutations in this gene are associated with Ehlers-Danlos syndrome types IV, and with aortic and arterial aneurysms. Two transcripts, resulting from the use of alternate polyadenylation signals, have been identified for this gene. [provided by R. Dalgleish, Feb 2008],
Specifications
| Size | 50μL/100μL |
| Target | Collagen III |
| Reactivity | Human |
| Applications | IHC, WB, IF, ELISA |
| MW(Calculated) | 150kD |
| MW(Observed) | 200kD |
| Host Species | Mouse |
| Isotype | IgG1,Kappa |
| Conjugate/Modification | Unmodified |
| Recommended Dilution Ratio | IHC 1:200-1000; WB 1:500-2000; IF 1:100-500; ELISA 1:1000-5000 / Note: For IHC, we suggest antigen retrieval with TE buffer pH 9.0 (Cat#WT1004) |
| Formulation | PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA |
| Purification | Recombinant Antibody expressed in animal component-free (ACF) media, purified via Protein G affinity chromatography. |
| Storage | -15°C to -25°C/1 year(Do not lower than -25°C) |
| Concentration | |
| Clonality | Monoclonal |
| Clone Number | RMM0118 |
| Immunogen | Synthesized peptide derived from human Collagen Type III AA range: 100-200 |
| Specificity | This antibody detects endogenous levels of COL3A1 protein. |
| Gene Name | COL3A1 |
| Protein Name | Collagen alpha-1(III) chain |
| Other Name | |
| Species | Human |
| Gene ID | 1281 |
| Uniprot | P02461 |
| Species.1 | |
| Gene ID.1 | |
| Uniprot.1 | |
| Species.2 | |
| Gene ID.2 | |
| Uniprot.2 | |
| Cellular Localization | Cytoplasmic |
| Tissue Expression | Colon carcinoma,Liver,Placenta,Skin fibroblast, |
| Signaling_pathway | Organismal Systems >> Immune system >> Platelet activation;Organismal Systems >> Endocrine system >> Relaxin signaling pathway |
| Research Areas | >>Platelet activation; / >>Relaxin signaling pathway; / >>AGE-RAGE signaling pathway in diabetic complications; / >>Protein digestion and absorption; / >>Amoebiasis; / >>Diabetic cardiomyopathy |
| Function | Disease:Defects in COL3A1 are a cause of Ehlers-Danlos syndrome type 3 (EDS3) [MIM:130020]; 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.,Disease:Defects in COL3A1 are a cause of susceptibility to aortic aneurysm abdominal (AAA) [MIM:100070]. 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.,Disease:Defects in COL3A1 are the cause of Ehlers-Danlos syndrome type 4 (EDS4) [MIM:130050]. 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.,Function:Collagen type III occurs in most soft connective tissues along with type I collagen.,online information:Collagen type III alpha-1 chain mutations,online information:Type-III collagen entry,PTM:O-linked glycan consists of a Glc-Gal disaccharide bound to the oxygen atom of a post-translationally added hydroxyl group.,PTM:Proline residues at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains.,similarity:Belongs to the fibrillar collagen family.,similarity:Contains 1 VWFC domain.,subunit:Trimers of identical alpha 1(III) chains. The chains are linked to each other by interchain disulfide bonds. Trimers are also cross-linked via hydroxylysines., |
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