本公司经销Cystatin B，胱抑素B/半胱氨酸蛋白酶抑制剂B抗体，克隆类型为polyclonal，宿主来源是Rabbit，Cystatin B胱抑素B/半胱氨酸蛋白酶抑制剂B抗体可应用于WB、elisa、IP、IF、IHC等实验，欢迎垂询订购！

货号：BY-5158R
英文名称：Anti-Cystatin B
中文名称：胱抑素B/半胱氨酸蛋白酶抑制剂B抗体
其他名称：CPI B; CST6; CSTB; Cystatin B; CYTB; EPM1; Liver thiol proteinase inhibitor; PME; STFB; CHROW21.
抗体来源：Rabbit
克隆类型：polyclonal
蛋白分子量：predicted molecular weight: 14kDa
纯化方法：affinity purified by Protein A
交叉反应：hu, mo, rat, pig
产品介绍：The Cystatin superfamily encompasses proteins that contain multiple Cystatin like sequences. Some of the members are active Cysteine protease inhibitors, while others have lost or perhaps never acquired this inhibitory activity. There are three inhibitory families in the superfamily, including the type 1 Cystatins （Stefins）, type 2 Cystatins ａnd Kininogens. Stefin B functions as an intracellular thiol protease inhibitor. The protein is able to form a dimer stabilized by noncovalent forces, inhibiting papain ａnd cathepsins l, h ａnd b. The protein is thought to play a role in protecting against the proteases leaking from lysosomes. Evidence indicates that mutated forms of this protein are responsible for the primary defects in patients with progressive myoclonic epilepsy.Subcellular Location : Cytoplasm. Nucleus.Involvement in disease: Defects in CSTB are the cause of progressive myoclonic epilepsy type 1 （EPM1） . EPM1 is an autosomal recessive disorder characterized by severe, stimulus-sensitive myoclonus ａnd tonic-clonic seizures. The onset, occurring between 6 ａnd 13 years of age, is characterized by convulsions. Myoclonus begins 1 to 5 years later. The twitchings occur predominantly in the proximal muscles of the extremities ａnd are bilaterally symmetrical, although asynchronous. At first small, they become late in the clinical course so violent that the victim is thrown to the floor. Mental deterioration ａnd eventually dementia develop.产品图片Antigen: bs-5158P, 0.2ug/100ul Primary: Antiserum, 1:500, 1:1000, 1:2000, 1:4000, 1:8000, 1:16000, 1:32000; Secondary: HRP conjugated Goat-Anti-Rabbit IgG（bs-0295G-HRP） at 1: 5000; TMB（C-0024） staining; Read the data in MicroplateReader by 450nm.