您好,欢迎来到试剂仪器网! [登录] [免费注册]
试剂仪器网
位置:首页 > 产品库 > 遗传性脉络膜缺乏症相关蛋白抗体
立即咨询
咨询类型:
     
*姓名:
*电话:
*单位:
Email:
*留言内容:
请详细说明您的需求。
*验证码:
 
遗传性脉络膜缺乏症相关蛋白抗体
本产品不向个人销售,仅用作科学研究,不用于任何人体实验及非科研性质的动物实验。

产品名称
Anti-CHM
Anti-CHM Rab escort protein Antibody
产品介绍
靶标:

CHM


产品别名:

DXS540; GGTA; HSD-32; REP-1; TCD; CHM; CHM Rab escort protein; CHM Rab escort protein; rab proteins geranylgeranyltransferase component A 1; CHM, Rab escort protein 1; choroideremia (Rab escort protein 1); 遗传性脉络膜缺乏症相关蛋白;


背景信息:
Rab escort protein 1 (REP1) also known as rab proteins geranylgeranyltransferase component A 1 is an enzyme that in humans is encoded by the CHM gene. It is mapped to Xq21.2. This gene encodes component A of the RAB geranylgeranyl transferase holoenzyme. In the dimeric holoenzyme, this subunit binds unprenylated Rab GTPases and then presents them to the catalytic Rab GGTase subunit for the geranylgeranyl transfer reaction. Rab GTPases need to be geranylgeranyled on either one or two cysteine residues in their C-terminus to localize to the correct intracellular membrane. Mutations in this gene are a cause of choroideremia; also known as tapetochoroidal dystrophy (TCD). This X-linked disease is characterized by progressive dystrophy of the choroid, retinal pigment epithelium and retina. Alternatively spliced transcript variants have been found for this gene.

宿主:Rbt
类型:Pab
同种型:IgG  
应用:WB
纯化方式:亲和纯化
偶联物:Unconjugated
性状:液体
存储溶液:参阅说明书
浓度:Batch dependent (Please refer to the vial label for the specific concentration.)
稀释比例: Optimal dilutions/concentrations should be determined by the end user           
储存:经常使用则4°C保存。-20°C保存不超过两年。避免反复冻融。
注意事项:仅供实验室使用。不适用于人类或动物的任何临床,治疗或诊断用途。不适合动物或人类食用。