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MABN52 Anti-REP-1 Antibody, clone 2F1

Anti-REP-1 Antibody, clone 2F1 is an antibody against REP-1 for use in WB, IP, IC, IH.

Anti-REP-1 Antibody, clone 2F1 MSDS (material safety data sheet) or SDS, CoA and CoQ, dossiers, brochures and other available documents.
Research Category: Neuroscience Research Sub-Category: Neurodegenerative Diseases Gene Symbol: CHM, REP-1, GGTA, TCD, HSD-32, REP1 UniProt Number: P24386
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MABN52
100 µg  
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Key Spec Table

Species ReactivityKey ApplicationsHostFormatAntibody Type
HWB, IP, ICC, IHCMPurifiedMonoclonal Antibody
Description
Catalogue NumberMABN52
DescriptionAnti-REP-1 Antibody, clone 2F1
Alternate Names
  • choroideremia (Rab escort protein 1)
  • choroideremia
  • Choroideraemia protein
  • Rab escort protein 1
  • Rab geranylgeranyltransferase component A
  • TCD protein
Background InformationRab 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.
References
Product Information
FormatPurified
Control
  • 293T cell lysate
PresentationPurified mouse monoclonal IgG1κ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
Quality LevelMQ100
Applications
ApplicationAnti-REP-1 Antibody, clone 2F1 is an antibody against REP-1 for use in WB, IP, IC, IH.
Key Applications
  • Western Blotting
  • Immunoprecipitation
  • Immunocytochemistry
  • Immunohistochemistry
Application NotesImmunohistochemistry 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
ImmunogenRecombinant protein corresponding to human REP-1.
EpitopeUnknown
Clone2F1
ConcentrationPlease refer to the Certificate of Analysis for the lot-specific concentration.
HostMouse
SpecificityThis antibody recognizes REP-1.
IsotypeIgG1κ
Species Reactivity
  • Human
Antibody TypeMonoclonal Antibody
Entrez Gene Number
Entrez Gene SummaryThis 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
  • CHM
  • REP-1
  • GGTA
  • TCD
  • HSD-32
  • REP1
Purification MethodProtein G Purified
UniProt Number
UniProt SummaryFUNCTION: 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
Physicochemical Information
Dimensions
Materials Information
Toxicological Information
Safety Information according to GHS
Safety Information
Product Usage Statements
Quality AssuranceEvaluated 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
  • Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
Storage and Shipping Information
Storage ConditionsStable for 1 year at 2-8°C from date of receipt.
Packaging Information
Material Size100 µg
Transport Information
Supplemental Information
Specifications
Global Trade Item Number
Catalogue Number GTIN
MABN52 04053252517365

Documentation

Anti-REP-1 Antibody, clone 2F1 SDS

Title

Safety Data Sheet (SDS) 

Anti-REP-1 Antibody, clone 2F1 Certificates of Analysis

TitleLot Number
Anti-REP-1, clone 2F1 2483254
Anti-REP-1, clone 2F1 - 2391170 2391170
Anti-REP-1, clone 2F1 - 2444683 2444683
Anti-REP-1, clone 2F1 - 1994921 1994921
Anti-REP-1, clone 2F1 - 2971252 2971252
Anti-REP-1, clone 2F1 - 3069694 3069694
Anti-REP-1, clone 2F1 - 3270757 3270757
Anti-REP-1, clone 2F1 - 3318358 3318358
Anti-REP-1, clone 2F1 - 3465955 3465955
Anti-REP-1, clone 2F1 - 3901385 3901385
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