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
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
Tested Application Dilution
FACS1:150 - 1:500
ICC/IF1:200 - 1:1000
IHC-P1:200 - 1:1000
WB1:500 - 1:2000
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.
Observed Size ~ 73 kDa

Properties

Form Liquid
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

GeneID: 1121 Human CHM

GeneID: 24942 Rat CHM

Swiss-port # P24386 Human Rab proteins geranylgeranyltransferase component A 1

Swiss-port # P37727 Rat Rab proteins geranylgeranyltransferase component A 1

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