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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, mouse

应用

WB, IHC, IF/ICC, Indirect ELISA

Choroideraemia protein, DXS540, GGTA, Rab escort protein 1, REP 1

缓冲液配方:  PBS Only
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, mouse samples.

经测试应用 WB, IHC, IF/ICC, Indirect ELISA Application Description
经测试反应性 human, mouse
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, DXS540, GGTA, Rab escort protein 1, REP 1
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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