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商品详细Hycult Biotech/Properdin,人,ELISA试剂盒| Hycult Biotech/HK334-01
Hycult Biotech/Properdin,人,ELISA试剂盒| Hycult Biotech/HK334-01
Hycult Biotech/Properdin,人,ELISA试剂盒| Hycult Biotech/HK334-01
商品编号: HK334-01
品牌: hycultbiotech
市场价: ¥12660.00
美元价: 7596.00
产地: 美国(厂家直采)
公司:
产品分类: 夹心法ELISA
公司分类: Sandwich_method_ELISA
联系Q Q: 3392242852
电话号码: 4000-520-616
电子邮箱: info@ebiomall.com
商品介绍
Properdin, or factor P, is a single-chain plasma glycoprotein (apparent molecular mass 52-55 kDa), consisting of six thrombospondin repeat sequences between short N- and C-terminal domains. In the blood it exists as a mixture of head-to-tail dimers, trimers and tetramers, with a preponderance of trimers.The protein is produced by a variety of leukocytes, including monocytes, T lymphocytes and neutrophils , but also by endothelial cells in which properdin synthesis and release are induced by shear stress.Properdin participates in the alternative pathway of complement activation together with C3 and factors B, D, I and H. It stabilizes the labile C3bBb which is deposited on immune complexes or foreign surfaces. This permits amplification of C3bBb formation in competition with catabolism of C3b by factor I, which uses factor H as a cofactor. The local amplification process leads to the creation of the alternative pathway C5 convertase, C3bBb3b, and initiates the terminal pathway of complement activation.Properdin prolongs the half-life of surface-bound C3bBb from 1½ minute to about 18 minutes. This is due to several effects: inhibition of C3b cleavage by factor I, increased affinity for factor B and inhibition of the dissociation of C3bBb into C3b and Bb. Properdin is consumed by binding to C3bBb, this binding showing an order of preference of tetramers over trimers over dimers, which is also the order of functional activity of the oligomeric forms.Deficiency or malfunction of the molecule may lead to severe impairment of alternative pathway activation, depending on the precise nature of the defect. One parameter of functional defect in the presence of measurable levels of protein is an impaired generation of the more active tetramer and trimer forms.The properdin gene is located on the short arm of the X chromosome, and congenital properdin deficiencies are therefore inherited as typical X-linked recessive traits.Three types of familial deficiency have been described: type 1 (or I) is characterized by serum with very low or absent properdin activity in hemolytic assays and <0.1 μg/ml="" immunoreactive="" protein;="" type="" 2="" (or="" ii)="" is="" characterized="" by="" low="" but="" detectable="" levels="" of="" immunoreactive="" protein="" (»2="" μg/ml)="" and="" impairment="" of="" some,="" but="" not="" all="" functional="" test,="" and="" type="" 3="" (or="" iii)-="" has="" normal="" levels="" of="" immunoreactive="" but="" dysfunctional="" protein.="" 10="" normal="" serum="" or="" plasma="" levels="" of="" properdin="" are="" variously="" quoted="" as="" about="" 5="" or="" 25="" µg/ml.="" differences="" in="" properdin="" levels="" between="" healthy="" and="" diabetic="" persons="">
品牌介绍
荷兰HyCult Biotechnology(HBT)位于荷兰乌登,从1994开始制造检测抗体和蛋白质,主要侧重与病理学诊断有关的全套产品研发,既有纯化的重组蛋白和多肽,也有单抗和多抗,是世界一流的先天免疫领域的研究试剂制造商。