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Key Specifications Table
| Species Reactivity | Key Applications | Host | Format | Antibody Type |
|---|---|---|---|---|
| H | WB, IP, ICC, IHC | M | Purified | Monoclonal Antibody |
| Description | |
|---|---|
| Catalogue Number | MABN52 |
| Description | Anti-REP-1 Antibody, clone 2F1 |
| Alternate Names |
|
| Background Information | Rab proteins are low molecular weight, ras-related GTPases that bind to a cell membrane’s cytoplasmic surface and function to regulate protein trafficking in both endocytic and secretory pathways. Rab escort protein (REP)-1 is a 653 amino acid protein belonging to the Rab GDI family of proteins. REP-1 binds to newly assembled unprenylated RAB proteins, escorts them to the Rab GGTases, and remains attached during and after the geranylgeranyl transfer reaction. REP proteins are composed of two conserved domains connected by a 150 amino acid insert. The multisheet domain I acts to assemble a Rab-binding platform. A globular, smaller, α-helical domain II, is thought to participate in membrane-protein interaction. Defects in the expression of REP-1 cause truncation or absence of the protein, and result in a disease called choroideremia (CHM), an X-linked blindness characterized by a progressive dystrophy of the retinal pigment epithelium, retina, and the choroid. |
| Product Information | |
|---|---|
| Format | Purified |
| Control |
|
| Presentation | Purified mouse monoclonal IgG1κ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide. |
| Quality Level | MQ100 |
| Applications | |
|---|---|
| Application | Anti-REP-1 Antibody, clone 2F1 is an antibody against REP-1 for use in WB, IP, IC, IH. |
| Key Applications |
|
| Application Notes | Immunohistochemistry Analysis: A previous lot of MABN52 was successfully used in IH, as reported by an independent laboratory (MacDonald, I.M., et al. (2005). Invest Ophthalmol Vis Sci. 46:E-Abstract 540). |
| Biological Information | |
|---|---|
| Immunogen | Recombinant protein corresponding to human REP-1. |
| Epitope | Unknown |
| Clone | 2F1 |
| Concentration | Please refer to the Certificate of Analysis for the lot-specific concentration. |
| Host | Mouse |
| Specificity | This antibody recognizes REP-1. |
| Isotype | IgG1κ |
| Species Reactivity |
|
| Antibody Type | Monoclonal Antibody |
| Entrez Gene Number |
|
| Entrez Gene Summary | 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. Alternative splicing results in multiple transcript variants encoding different isoforms. |
| Gene Symbol |
|
| Purification Method | Protein G Purified |
| UniProt Number |
|
| UniProt Summary | FUNCTION: Binds unprenylated Rab proteins, presents it to the catalytic Rab GGTase dimer, and remains bound to it after the geranylgeranyl transfer reaction. The component A is thought to be regenerated by transferring its prenylated Rab back to the donor membrane. SUBUNIT STRUCTURE: Monomer. Interacts with RAB1B; the interaction is required for RAB1B prenylation. Interacts with RGGT (By similarity). INVOLVEMENT IN DISEASE: Defects in CHM are the cause of choroideremia (CHM) [MIM:303100]; also known as tapetochoroidal dystrophy (TCD). CHM is a common form of X-linked blindness characterized by progressive dystrophy of the choroid, retinal pigment epithelium and retina. SEQUENCE SIMLARITIES: Belongs to the Rab GDI family. |
| Molecular Weight | ~83 kDa observed |
| Product Usage Statements | |
|---|---|
| Quality Assurance | Evaluated by Western Blot in 293T cell lysate. Western Blot Analysis: 0.5 µg/mL of this antibody detected REP-1 on 10 µg of 293T cell lysate. |
| Usage Statement |
|
| Storage and Shipping Information | |
|---|---|
| Storage Conditions | Stable for 1 year at 2-8°C from date of receipt. |
| Packaging Information | |
|---|---|
| Material Size | 100 µg |