首页 > Hycult Biotech > Hycult Biotech/Alpha-1-antitrypsin, Human, clone 2C1 | Hycult Biotech/HM2289-500UG

提醒：代购产品，无质量问题不接受退换货，下单前请仔细核对信息。下单后请及时联系客服核对商品价格，订单生效后再付款。

Hycult Biotech/Alpha-1-antitrypsin, Human, clone 2C1 | Hycult Biotech/HM2289-500UG

价格：

登录查看

自营商城

解放采购

正品保障

及时交付

厂家直采

一站服务

货号：

HM2289-500UG

品牌：

Hycult Biotech

会员服务：

尊享会员价

贵宾专线

运费优惠

闪电退款

福利优惠

上门换新

友情提示

以上价格仅为参考，请联系客服询价。

免费咨询热线

4000-520-616

产品说明

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.

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。