ARG42590
anti-CHM / REP1 antibody
anti-CHM / REP1 antibody for Western blot,IHC-Formalin-fixed paraffin-embedded sections,ICC/IF,Flow cytometry and Human,Mouse,Rat
Overview
Product Description | Rabbit Polyclonal antibody recognizes CHM / REP1 |
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Tested Reactivity | Hu, Ms, Rat |
Tested Application | FACS, ICC/IF, IHC-P, WB |
Host | Rabbit |
Clonality | Polyclonal |
Isotype | IgG |
Target Name | CHM / REP1 |
Antigen Species | Human |
Immunogen | Synthetic peptide corresponding to a sequence of Human CHM / REP1. (QDQILENEEAIALSRKDKTIQHVEVFCYASQDLHED) |
Conjugation | Un-conjugated |
Alternate Names | Rab proteins geranylgeranyltransferase component A 1; GGTA; TCD protein; HSD-32; DXS540; REP-1; TCD; Rab escort protein 1; Choroideremia protein |
Application Instructions
Application Suggestion |
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Application Note | IHC-P: Antigen Retrieval: Heat mediation was performed in Citrate buffer (pH 6.0) for 20 min. * The dilutions indicate recommended starting dilutions and the optimal dilutions or concentrations should be determined by the scientist. |
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Observed Size | ~ 73 kDa |
Properties
Form | Liquid |
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Purification | Affinity purification with immunogen. |
Buffer | 0.2% Na2HPO4, 0.9% NaCl, 0.05% Sodium azide and 4% Trehalose. |
Preservative | 0.05% Sodium azide |
Stabilizer | 4% Trehalose |
Concentration | 0.5 - 1 mg/ml |
Storage Instruction | For continuous use, store undiluted antibody at 2-8°C for up to a week. For long-term storage, aliquot and store at -20°C or below. Storage in frost free freezers is not recommended. Avoid repeated freeze/thaw cycles. Suggest spin the vial prior to opening. The antibody solution should be gently mixed before use. |
Note | For laboratory research only, not for drug, diagnostic or other use. |
Bioinformation
Database Links |
Swiss-port # P24386 Human Rab proteins geranylgeranyltransferase component A 1 Swiss-port # P37727 Rat Rab proteins geranylgeranyltransferase component A 1 |
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Gene Symbol | CHM |
Gene Full Name | choroideremia (Rab escort protein 1) |
Background | 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. [provided by RefSeq, Mar 2016] |
Function | Substrate-binding subunit of the Rab geranylgeranyltransferase (GGTase) complex. Binds unprenylated Rab proteins and presents the substrate peptide to the catalytic component B composed of RABGGTA and RABGGTB, 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. Besides, a pre-formed complex consisting of CHM and the Rab GGTase dimer (RGGT or component B) can bind to and prenylate Rab proteins; this alternative pathway is proposed to be the predominant pathway for Rab protein geranylgeranylation. [UniProt] |
Cellular Localization | Cytoplasm, cytosol. [UniProt] |
Calculated MW | 73 kDa |