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商品详细Hycult Biotech/Alpha-1-antitrypsin, Human, clone 2C1 | Hycult Biotech/HM2289
Hycult Biotech/Alpha-1-antitrypsin, Human, clone 2C1  | Hycult Biotech/HM2289
Hycult Biotech/Alpha-1-antitrypsin, Human, clone 2C1 | Hycult Biotech/HM2289
商品编号: HM2289
品牌: hycultbiotech
市场价: ¥6980.00
美元价: 4188.00
产地: 美国(厂家直采)
公司:
产品分类: 单克隆抗体
公司分类: monoclonal_antibody
联系Q Q: 3392242852
电话号码: 4000-520-616
电子邮箱: info@ebiomall.com
商品介绍
The mouse monoclonal antibody clone 2C1 recognizes polymeric forms of human alpha-1-antitrypsin. Alpha-1-antitrypsin is the most abundant circulating protease inhibitor. Serpinopathies are conformational diseases characterized by the polymerization and intracellular retention of members of the serine protease inhibitor or serpin superfamily of proteins.1 The best known is a1-antitrypsin deficiency, with the most common severe deficiency allele being the Z mutation (Glu342Lys). The severe Z deficiency allele (Glu342Lys) causes the protein to undergo a conformational transition and form ordered polymers that are retained within hepatocytes. This causes neonatal hepatitis, cirrhosis, and hepatocellular carcinoma. Clone 2C1 recognizes polymers formed by Zα1-antitrypsin in vivo. It also recognizes polymers formed by the Siiyama (Ser53Phe) and Brescia (Gly225Arg) mutants, and the novel His334Asp shutter domain mutant of a1-antitrypsin that is associated with prolonged neonatal jaundice in a 6-week-old boy. These data show that Z and shutter domain mutants form polymers with a shared epitope.
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。