产品类别

 兔抗人、大、小鼠FBN1抗体

产品价格

 0.1ml/480元  0.2ml/650元  

产品标准

 浓缩液(1mg/1ml) 亲和纯化抗体

产品应用

 WB=1:100-500 Elisa =1:200-1000 IP=1:20-100 IHC=1:100-500    （石蜡切片需做抗原修复）

 

产品介绍

 Fibrillin-1广泛存在于皮肤、肺、肾、血管、软骨、睫装小体等，原纤维蛋白1在产生强而韧性的组织中起重要作用，是组织连结重要的蛋白。
 Fibrillin is the major constitutive element of extracellular microfibrils and has widespread distribution in both elastic and nonelastic connective tissue throughout the body. The cDNA was identified in 1991 and was mapped coincident with the locus for Marfan syndrome. Subsequent studies confirmed that mutations in the Fibrillin 1 gene are the major cause of Marfan syndrome. The connective tissue protein has a widespread distribution in the connective tissue matrices of skin, lung, kidney, vasculature, cartilage, tendon, muscle, cornea, and ciliary zonule.
 mol wt:Fibrillin 1 is an extracellular acidic protein with a high cysteine content and an extended threadlike shape with mosaic composition of different types of extracellular modules. Most of the fibrillin molecule is contributed by 47 epidermal growth factor like (EGF like) repeats; 43 of them have a consensus sequence for calcium binding (cb). Other modules including 8 cysteine motifs (seven), hybrid motifs (two), and unique domains (three) are interspersed throughout the molecule. Fibrillin 1 contains a proline rich region close to the N terminus that is replaced by a glycine rich region in Fibrillin 2. The fibrillins are found throughout the connective tissue as integral components of extended fibrils.