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Anti-REP-1, clone 2F1 (mouse monoclonal)

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货号：MABN52

品牌：Millipore 密理博

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重要规格表

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
  • choroideremia (Rab escort protein 1)
  • choroideremia
  • Choroideraemia protein
  • Rab escort protein 1
  • Rab geranylgeranyltransferase component A
  • TCD protein
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.
References
Product Information
Format Purified
Control
  • 293T cell lysate
Presentation Purified mouse monoclonal IgG1κ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
Applications
Application Anti-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 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
  • Human
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
  • CHM
  • REP-1
  • GGTA
  • TCD
  • HSD-32
  • REP1
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
Physicochemical Information
Dimensions
Materials Information
Toxicological Information
Safety Information according to GHS
Safety Information
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
  • 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 Conditions Stable for 1 year at 2-8°C from date of receipt.
Packaging Information
Material Size 100 µg
Transport Information
Supplemental Information
Specifications

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