产品概述
| 产品名称（Product Name） | Collagen III(Q76)Mouse Monoclonal Antibody |
| 描述（Description） | Mouse Monoclonal Antibody |
| 宿主（Host） | Mouse |
| 应用（Application） | IHC-P,IF-P,IF-F,ICC/IF |
| 种属反应性（Reactivity） | Human,Mouse,Rat |
产品性能
| 偶联物（Conjugation） | Unconjugated |
| 修饰（Modification） | Unmodified |
| 同种型（Isotype） | IgG |
| 克隆（Clonality） | Monoclonal |
| 形式（Form） | Liquid |
| 存放说明（Storage） | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| 储存溶液（Buffer） | PBS, pH 7.4, containing 0.5%BSA, 0.02% New type preservative N as Preservative and 50% Glycerol. |
| 纯化方式（Purification） | Affinity purification |
免疫原
| 基因名（Gene Name） | COL3A1 |
| 别名（Alternative Names） | COL3A1; Collagen alpha-1(III) chain |
| 基因ID（Gene ID） | 1281 |
| 蛋白ID（SwissProt ID） | P02461 |
产品应用
| 稀释比（Dilution Ratio） | IF-P/IF-F/ICC/IF 1:200, IHC-P 1:50-300 |
| 蛋白分子量（Molecular Weight） | 138kDa |
研究背景
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],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.,
研究领域
Focal adhesion;ECM-receptor interaction;
关键字： COL3A1;Collagen;III(Q76)Mouse;Monoclonal;Antibody;一抗
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