Validation Data Gallery
Tested Applications
Recommended dilution
| Application | Dilution |
|---|---|
| It is recommended that this reagent should be titrated in each testing system to obtain optimal results. | |
Product Information
26251-1-PBS targets CHM in WB, IHC, IF/ICC, Indirect ELISA applications and shows reactivity with human samples.
| Tested Reactivity | human |
| Host / Isotype | Rabbit / IgG |
| Class | Polyclonal |
| Type | Antibody |
| Immunogen |
CatNo: Ag23166 Product name: Recombinant human CHM protein Source: e coli.-derived, PGEX-4T Tag: GST Domain: 69-110 aa of BC063522 Sequence: VSDSPVWQDQILENEEAIALSRKDKTIQHVEVFCYGRSTLLL 相同性解析による交差性が予測される生物種 |
| Full Name | choroideremia (Rab escort protein 1) |
| Observed molecular weight | 95 kDa |
| GenBank accession number | BC063522 |
| Gene Symbol | CHM |
| Gene ID (NCBI) | 1121 |
| RRID | AB_3669530 |
| Conjugate | Unconjugated |
| Form | |
| Form | Liquid |
| Purification Method | Antigen affinity purification |
| UNIPROT ID | P24386 |
| Storage Buffer | PBS only{{ptg:BufferTemp}}7.3 |
| Storage Conditions | Store at -80°C. |
Background Information
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).