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BN42185R-50ul
50ul
￥1486.00
交叉反應(yīng)：Human,Rat(predicted:Mouse,Cow) 推薦應(yīng)用：WB,IHC-P,IHC-F,IF,ELISA
BN42185R-100ul
100ul
￥2360.00
交叉反應(yīng)：Human,Rat(predicted:Mouse,Cow) 推薦應(yīng)用：WB,IHC-P,IHC-F,IF,ELISA
BN42185R-200ul
200ul
￥3490.00
交叉反應(yīng)：Human,Rat(predicted:Mouse,Cow) 推薦應(yīng)用：WB,IHC-P,IHC-F,IF,ELISA
產(chǎn)品描述
| 英文名稱 | Collagen III |
| 中文名稱 | Ⅲ型膠原蛋白/膠原蛋白3/3型膠原蛋白抗體 |
| 別 名 | COL 3A1; COL3A1; Collagen alpha 1(III) chain; Collagen III alpha 1 chain precursor; Collagen III alpha 1 polypeptide; Collagen type III alpha 1 (Ehlers Danlos syndrome type IV autosomal dominant); Collagen type III alpha 1; Collagen type III alpha; EDS4A; Ehlers Danlos syndrome type IV, autosomal dominant; Fetal collagen; Type III collagen; CO3A1_HUMAN; Collagen alpha-1(III) chain; Type III collagen; type III preprocollagen alpha 1 chain.. |
| 研究領(lǐng)域 | 細(xì)胞生物 免疫學(xué) 信號(hào)轉(zhuǎn)導(dǎo) |
| 抗體來(lái)源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | Human, Rat, (predicted: Mouse, Cow, ) |
| 產(chǎn)品應(yīng)用 | WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蠟切片需做抗原修復(fù)） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 117kDa |
| 細(xì)胞定位 | 細(xì)胞外基質(zhì) 分泌型蛋白 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Collagen alpha 1(III) chain:801-900/1466 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲(chǔ) 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. |
| PubMed | PubMed |
| 產(chǎn)品介紹 | The extensive family of COL gene products (collagens) is composed of several chain types, including fibril-forming interstitial collagens (types I, II, III and V) and basement membrane collagens (type IV), each type containing multiple isoforms. Collagens are fibrous, extracellular matrix proteins with high tensile strength and are the major components of connective tissue, such as tendons and cartilage. All collagens contain a triple helix domain and frequently show lateral self-association in order to form complex connective tissues. Several collagens also play a role in cell adhesion, important for maintaining normal tissue architecture and function. 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. Function: Collagen type III occurs in most soft connective tissues along with type I collagen. 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. Subcellular Location: Secreted, extracellular space, extracellular matrix. 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. 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. 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. 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. Similarity: Belongs to the fibrillar collagen family. Contains 1 fibrillar collagen NC1 domain. Contains 1 VWFC domain. SWISS: P02461 Gene ID: 1281 Database links: Entrez Gene: 1281 Human Entrez Gene: 12825 Mouse Omim: 120180 Human SwissProt: P02461 Human SwissProt: P08121 Mouse Unigene: 443625 Human Unigene: 249555 Mouse Unigene: 3247 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
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