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CHM Polyclonal antibody, PBS Only

CHM Polyclonal Antibody for WB, IHC, IF/ICC, Indirect ELISA
Cat No. 26251-1-PBS

产品说明书

宿主/亚型

Rabbit / IgG

种属反应性

human

应用

WB, IHC, IF/ICC, Indirect ELISA

Choroideraemia protein, REP1, REP 1, Rab escort protein 1, GGTA

缓冲液配方: 
偶联物:  Unconjugated
规格价格库存


产品信息

26251-1-PBS targets CHM in WB, IHC, IF/ICC, Indirect ELISA applications and shows reactivity with human samples.

经测试应用 WB, IHC, IF/ICC, Indirect ELISA Application Description
经测试反应性 human
免疫原

CatNo: Ag23166

Product name: Recombinant human CHM protein

Source: e coli.-derived, PGEX-4T

Tag: GST

Domain: 69-110 aa of BC063522

Sequence: VSDSPVWQDQILENEEAIALSRKDKTIQHVEVFCYGRSTLLL

种属同源性预测
宿主/亚型 Rabbit / IgG
抗体类别 Polyclonal
产品类型 Antibody
全称 choroideremia (Rab escort protein 1)
别名 Choroideraemia protein, REP1, REP 1, Rab escort protein 1, GGTA
观测分子量 95 kDa
GenBank蛋白编号BC063522
基因名称 CHM
Gene ID (NCBI) 1121
RRIDAB_3669530
偶联类型 Unconjugated
形式Liquid
纯化方式Antigen affinity purification
UNIPROT IDP24386
储存缓冲液 PBS only, pH 7.3.
储存条件Store at -80°C.
The product is shipped with ice packs. Upon receipt, store it immediately at -80°C

背景介绍

CHM (Choroideremia protein) is also named as Rab escort protein 1 (REP-1), TCD and Rab proteins geranylgeranyltransferase component A 1. Choroideremia (CHM) is an X-linked recessive eye disease that is caused by null mutations in REP-1, a gene involved in the regulation of Rab GTPases and intracellular vesicular transport (PMID: 9067750, PMID: 16936131). REP-1 is involved in trafficking of Rab proteins in the cell. And REP-1 binds newly synthesized Rab proteins and presents them to the catalytic site of RabGGTase (PMID: 19427510). RAB-27 in several neurons, such as motor neurons at neuromuscular junctions, requires REP-1 for normal synaptic transmission responsible for aldicarb sensitivity (PMID: 19090809). REP-1 acts as a component of the protein complex. There are two similar Rab escort proteins (75% sequence identity): REP-1 and REP-2. A 95-kDa protein (previously designated component A and now called REP-1) is present (PMID: 8294464, PMID: 17140818).

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