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Collagen III (2X2) Rabbit Monoclonal Antibody  Cite:0    分享

货号: IM68936
复制产品信息
  • 种属:H,M,R
  • 用途:WB,ELISA,IP,IHC-P,IF-P,IF-F,IF-ICC
规格:
50μl 100μl
单位:
单价:¥1300.00
产品简介
储存与保存
注意事项
产品简介
  • 产品货号
    IM68936
  • 产品名称
    Collagen III (2X2) Rabbit Monoclonal Antibody
  • 类别
    抗体产品
  • 基因名称
    COL3A1
  • 蛋白名称
    Collagen alpha-1(III) chain
  • Clonality
    Monoclonal
  • 推荐应用
    WB,IHC-P,IF-P,IF-F,IF-ICC,IP,ELISA
  • 反应种属
    Human,Mouse,Rat
  • 存储缓冲液
    PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA
  • Human Gene ID
    1281
  • Human Swissprot No.
    P02461
  • 稀释度
    IHC-P 1:20-100,WB 1:1000-5000,IF-P/IF-F/IF-ICC 1:200-1000,ELISA 1:5000-20000,IP 1:50-200
  • 参考分子量
    150kDa
  • 预测分子量
    150kDa
  • 宿主
    Rabbit
  • 同种型
    Monoclonal, rabbit, IgG, Kappa
  • 背景介绍
    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].
  • 信号通路
    Focal adhesion;ECM-receptor interaction.
  • 功能
    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.
  • 纯化
    Protein A
储存与保存

1.保存:-20℃

2.有效期:1年

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