Anti-UBE1/E1 Ubiquitin Activating Enzyme泛素激活酶E1抗体

本公司提供科研UBE1/E1 Ubiquitin Activating Enzyme泛素激活酶E1抗体，抗体质量可靠，订购UBE1/E1 Ubiquitin Activating Enzyme泛素激活酶E1抗体请联系在线客服或者销售人员。
抗体参数如下>>>>
中文名称：泛素激活酶E1抗体
英文名称：Anti-UBE1/E1 Ubiquitin Activating Enzyme
货号：bs-6835R
抗体来源：兔
克隆类型：多克隆
蛋白分子量：predicted molecular weight: 118kDa
纯化方法：affinity purified by Protein A
交叉反应：hu, mo, rat, Rb, dog
测试应用：ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500
（石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
产品背景介绍：Activates ubiquitin by first adenylating its C-terminal glycine residue with ATP, and thereafter linking this residue to the side chain of a cysteine residue in E1, yielding an ubiquitin-E1 thioester and free AMP.Involvement in disease: Defects in UBA1 are the cause of spinal muscular atrophy X-linked type 2 (SMAX2) ; also known as X-linked lethal infantile spinal muscular atrophy, distal X-linked arthrogryposis multiplex congenita or X-linked arthrogryposis type 1 (AMCX1). Spinal muscular atrophy refers to a group of neuromuscular disorders characterized by degeneration of the anterior horn cells of the spinal cord, leading to symmetrical muscle weakness and atrophy. SMAX2 is a lethal infantile form presenting with hypotonia, areflexia, and multiple congenital contractures.